Vas deferens ectopia is a rare congenital anomaly frequently associated with anorectal abnormalities and hypospadias. We present a Currarino syndrome case with an ectopic vas deferens terminating in a distal retroiliac ureter. A left vasectomy, ureteral decussation over the iliac vessels, and a Cohen's-type ureteral reimplantation were performed. The objectives are to preserve renal function, prevent epididymitis, and preserve fertility. There is no evidence in the literature that recommends surgical correction of the vas deferens. Microsurgical epididymal sperm aspiration or testicular sperm extraction combined with intracytoplasmic sperm injection might be a viable strategy for these affected individuals.