The aim of the study was to detect the incidence and types of genital and renal duct anomalies associated with congenital absent vas deferens (CAVD). In 200 males with CAVD, the demographic characteristics, physical examination findings were evaluated. Scrotal ultrasonography and transrectal ultrasonography (TRUS) were used as the diagnostic methods for evaluating unilateral or bilateral CAVD with associated abnormalities or agenesis of the testes, epididymes, seminal vesicles and prostate. Abdominal ultrasound was performed to detect any associated renal anomalies. There were a total of 111 CBAVD and 89 CUAVD males. Eight cases (8.98%) of CUAVD were associated with contralateral cryptorchidism. In most cases there were agenesis in the epididymal body and tail and seminal vesicles. Different types of renal anomalies (32.50%) especially renal agenesis were observed mainly in cases of left CUAVD and were predominately on the left hand side. An important implication of our study is the importance of requesting ultrasound of males with CAVD to discover any associated anomalies especially renal agenesis that may be ignored by many physicians.