A 38-year-old man, with no past history of interest, came to the urology clinic requesting a vasectomy. The physical examination revealed a normal male subject, with penis, testicles and epididymis of normal size and consistency. The right vas deferens was clearly palpable. This was not the case with the left vas deferens which could not be palpated within the spermatic cord. Rectal examination was unremarkable. In view of the suspicion of vas deferens agenesis, a transrectal ultrasound was performed, in which the left seminal vesicle was not visualised. IVUS revealed the complete absence of the left kidney. Subsequently, an MRI confirmed the absence of the left seminal vesicle, vas deferens and ureter. The patient was scheduled for vasectomy which was performed uneventfully on the right side. Control seminograms performed three months after the operation revealed complete azoospermia, confirming the clinical suspicion of deferential agenesis associated with renal and homolateral seminal vesicle agenesis. The patient's first-degree relatives (two sons, a male brother and his two sons) were advised to undergo an ultrasound study, given the potential autosomal dominant transmission of renal agenesis, and both kidneys were found to be present in all of them. The patient's sweat test was also negative.