Primitive neuroectodermal tumor (PNET)/Ewing's sarcoma tumors are a group of highly malignant tumors composed of small, round cells showing neuroectodermal differentiation that commonly affects soft tissue and bone. PNET involving the genitourinary system is rare and PNET of the penile urethra is rarer still. It exhibits a highly aggressive biological behavior with poor prognosis. We report a case of a 27-year-old male presenting with penile swelling and difficulty in passing urine. Examination revealed a firm penile urethral mass. Pathological and immunohistochemical results of the specimen obtained from urethroscopic biopsy followed by total penectomy confirmed the diagnosis of PNET of the urethra. He received combination chemotherapy: ifosfamide and etoposide (IE) + vincristine, Adriamycin, and cyclophosphamide (VAC). In conclusion, PNET has to be considered in the differential diagnosis of a penile urethral mass in young patients.