Unilateral renal agenesis with an absence of the seminal vesicle, epididymis and ductus deferens is rare and is the result of a developmental disorder of the mesonephric or Wolffian duct. We report the case of a 22-year-old man who presented with testicular pain on the left side of 3 weeks' duration. During the clinical investigation of the scrotum a nonpalpable ductus deferens on the left side was found incidentally. As a result of the urological ultrasound the diagnosis of renal, epididymal, seminal vesicle and ductus deferens agenesis on the left was confirmed. As a vascular variety the CT demonstrated 2 renal veins and 2 renal arteries on the right originating from the superior mesenteric artery together with the right hepatic artery. The testicular artery was placed on both sides. Further diagnostic investigations including a spermiogram, hormone analysis and kidney function tests were normal. Congenital urogenital malformations can be found in various combinations even in adults. Unilateral absence of the vas deferens during clinical examination should alert the clinician to an underlying renal, seminal vesicle and epididymal anomaly; further urological investigation is mandatory. A genetic investigation of the CFTR gene is not necessary in the absence of both ductus deferentes with renal agenesis.
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