Vas deferens ectopia is a rare congenital anomaly which is frequently associated with anorectal abnormalities. There have been 20 cases up to date with an ectopic vas deferens inserting into the ureter. Out of these 6 had a bilateral insertion and another 4 cases with an ectopic vas deferens inserting directly into the bladder, out of which 1 had a bilateral insertion. There is an embryological hypothesis which may explain this rare condition that may result from a possible underlying defect of the proximal vas precursor segment of the Wolffian duct. Herein, we present a case of an ectopic vas deferens terminating in the urinary bladder and accompanied by ipsilateral renal agenesis. This is the second case report in the world literature.