We report the case of a young man who presented with numerous episodes of ipsilateral epididymitis. Selected imaging studies with consideration of urogenital embryology lead to the rare diagnosis of a dysplastic kidney with ureteral ectopia in the seminal vesicle. After nephroureterectomy and vesiculectomy, convalescence was uneventful. No specific symptoms, equivocal diagnostic findings and the small number of patients limit the surgical experience in diseases of the seminal vesicle. Suprainguinal extravesical extirpation, however, appears to be an excellent operative approach in cases of unilateral seminal vesicle cysts.