Polyorchidism is a very rare anomaly of the genitourinary tract and about 100 cases have been reported in the literature. Although polyorchidism is associated with undescended testis, inguinal hernia, testicular torsion, hydrocele, epididymitis, varicocele and cancer, the majority of the patients are asymptomatic and present with painless groin or testicular masses. We describe a case of polyorchidism in a 19-year-old man with varicocele and review the literature and current management of polyorchidism.