A case of perforated complete transverse vaginal septum in the upper third of the vagina in a patient presenting at 19 years of age for evaluation of primary infertility. Diagnosis was made by hysterosalpingography. The patient was treated surgically and conceived 2 months after the procedure. The finding of transverse vaginal septum in an asymptomatic infertility patient is highly unusual. We reviewed the literature since 1966 and summarized 73 cases. The review confirms the benign characteristics of this rare anomaly and supports the embryonic origin of the transverse septum from the vaginal plate.