Congenital bilateral absence of the vas deferens (CBAVD) is a rare obstructive anomaly contributing to male factor infertility. Various congenital anomalies associated with CBAVD involve the seminal vesicles and epididymis. Physical examinations are often not reliable. However, transrectal ultrasonography (TRUS) can distinguish seminal vesicle and epididymal anomalies. In this clinical report, a rare case of CBAVD without seminal vesical anomalies is presented. PE and TRUS revealed no remarkable findings. The patient underwent vaso-epididymal anastomosis for the seminal tract obstruction and was accidentally diagnosed with CBAVD. Although ultrasonography is a reliable approach, surgical methods are critical for the diagnosis of CBAVD.