A triad of unilateral renal agenesis, ipsilateral seminal vesicle cyst and ipsilateral ejaculatory duct obstruction has been called Zinner syndrome since its first description in 1914 by Zinner. It is a very rare congenital abnormality of the male genitourinary tract due to abnormal embryologic development of the Wolffian ducts. There have been several case reports from different parts of the world about the clinical and imaging findings of these anomaly. In this case report, we present the first case report of Zinner syndrome in a 28-year-old male Ethiopian patient.