This study considers a unilateral renal agenesia associated with agenesia of the epididymis body and tail and the vas deferens and non-palpable left testicle in a 20-month-year-old patient. During laparoscopic procedure, the testicle was positioned at approximately 5 cm above the inguinal ring. The size was appropriate for the age and the head of the epididymis was situated in its normal position. The decision was made to perform the first step of the Fowler-Stephens’ surgery and the patient presented a good evolution. The association of male duct system agenesia with unilateral renal agenesia in a patient with cryptorchidism diagnosed by laparoscopy is an extremely rare event, however generally in these cases the testicle is of normal size, presents unaltered hormonal function, and must be preserved.