the penis agenesis ( pa ) is an extremely rare genitourinary anomaly with an incidence of about one in 30  million births . it is believed to result from the absence or a failure in the development of genital tubercule . since it was originally described by imminger in 1853 , approximately 100 cases have been described worldwide . second one is the complex form which is often incompatible with life because of accompanying congenital anomalies . more than half of these patients have associated anomalies including genitourinary ( 54% ) and gastrointestinal tract anomalies and developmental defects of caudal axis . skoog and bellman   have classified these patients based on the relationship between the anal sphincter and the ectopic urethral meatus . three variations were described by them : the first one is postsphincteric form with anterior perianal urethra , the second one is presphincteric urethrorectal fistula and the third one is urethral atresia with vesicorectal fistula . urethral opening could be either over pubis or at any part of perineum or most frequently in anterior wall of the rectum . other anomalies and death are seen related with more proximal opening of the bladder outlet . historically , gender reassignment at infantile period of life has been considered as the most appropriate choice for these patients . surgical intervention included urethral transposition and bowel vaginoplasty via posterior sagittal approach plus scrotoplasty and orchiectomy . although adequate outcomes have been reported , the majority of these patients demonstrated male - typical shift in psychosocial and pyschosexual development in long time follow up . a 22 year - old patient was referred to us due to absence of penis and urination through rectum . physical examination revealed agenesis of penis , normal scrotum , bilateral normally positioned testis and vas deferens , well male secondary sexual characteristics , normally located and at tone anus and anal sphincter . 1 ) . ultrasonography showed the absence of left kidney , superior segment of ureter and prostate gland . left ureterocele , increased bladder wall thickness due to persistant urinary tract infections and dilated right ureter were also seen . buccal smear was consistent with male genotype , and chromosomal studies revealed a normal karyotype 46xy . cystouretrogram performed through rectosigmoidoscopy showed normal appearing bladder , a vesicorectal fistula opening to the anterior wall of the rectum , left ureterocele and grade 2 right vesicoureteral reflux ( fig . 2 ) . 99mtc - dimercaptosuccinyl acid ( dmsa ) renal scyntigraphy , computerized tomography ( ct ) , urography and magnetic resonance imaging ( mri ) were performed in order to plan the surgical intervention . although the patient and his parents were informed about the masculinizing operation , they refused any surgical intervention because of their religious beliefs . we acquainted the patient with the possible health problems and invited him to regular follow up . the embriology of this rare anomally has been described as a result of the genital tubercule failure to develop with incomplete seperation of the urogenital sinus from the hindgut by the urorectal septum . the classic pa diagnosis includes 46 xy normal male karyotype , complete absence of the penis , a normal scrotum , normal and frequently undescended testis . urethral opening can be located in the perineal area , mostly inside a foreskin appearing skin tag or the anterior wall of the rectum . pa must be differantiated from rudimentary penis , micropenis , concealed penis , intaruterin amputation of penis and disorders of sexual development . these infants with pa underwent gender reassignment surgery , including bilateral orchiectomy , urethral transposition , vaginal replacement and labial construction . although adequate outcomes have been reported , the majority of these patients demonstrasted male typical shift in psychosocial and pyschosexual development in a long time follow up . since the patient 's gender identity is formed after the second year of life several authors have advised to perform masculinizing operations , in order not to disturb the patient and his parents psychologically as we have advised in our case . in conclusion , treatment of pa presents many challenges and it involves multidisciplinary approach . the team should include a urologist , a pediatrician , an endocrinologist , a geneticist , and a mental health expert . , they should be informed about the devastating outcomes of this anomally and invited to regular follow up controls by the urologist . we declare that the authors have obtained informed consent from the patient to report this case .
the penis as a component of external genitalia , takes part in fertility , urinary and psychosexual structure of males with its complex character . 
 we report a case of penis agenesis with associated left renal agenesis , left superior segment ureteral agenesis , prostate agenesis , left ureterocele , right vesicoureteral reflux and high urethrorectal communication above the rectal sphincter . 
 the patient refused any surgical intervention because of his religious beliefs .