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This article discusses scrotal ultrasonic features of patients with a congenital bilateral absence of the vas deferens (CBAVD).Thirty-six patients with CBAVD were examined to obtain image characteristics of the bilateral testicle and epididymis by ultrasound. Each patient received a pathological examination of the sperm by percutaneous epididymal biopsy.Scrotal ultrasonic features were as follows: (1) testicular volumes were normal in all the 36 patients; (2) 21% (15/72) of the heads of epididymis, 67% (48/72) of the heads and bodies of epididymis, and 12% (9/72) of the heads, bodies, and tails of epididymis could be shown in the 72 epididymides of the 36 patients; (3) cystic or tubular dilation of the epididymis was obvious in all 72 abnormal epididymides; and (4) bilateral vas deferens could not be found in these patients. Johnsen's grading of sperm in all the 36 patients received a score of 7 or higher (scale of 10, lower numbers denote greater dysfunction).Ultrasonography images of the scrotum revealed characteristics that were commonly present in patients with CBAVD. Therefore, this method is helpful to diagnose CBAVD by identifying the features of epididymis structural abnormalities and cystic or tubular dilation of the epididymis.