Congenital seminal vesicle cysts associated with renal agenesis are uncommon, but are currently detected more frequently with the use of sectional imaging procedures. Approximately 200 cases have been reported. The unique feature of our case is the combination of this disorder with an ipsilateral undescended testis. Our patient underwent partial vesiculectomy, in which the cyst was removed and the seminal vesicle remnant with its vas deferens was preserved. A review of the infertile cases and the impact of surgical treatment on fertility are discussed. Features that render partial vesiculectomy applicable and the potential effect of this procedure on fertility are highlighted.