Transverse vaginal septum is a rare condition that results from abnormalities in the vertical fusion between the vaginal components of the Mullerian ducts and the urogenital sinus. Associated menouria as a result of a fistulous connection of the vagina with the bladder is even rarer. A case is presented of a 22 year old unmarried woman who in addition to presenting with primary amenorrhea, complete transverse vaginal septum and cyclic hematuria, also had unilateral renal agenesis and a corrected congenital imperforate anus. A surgical correction is described as well as simple measures during follow-up that ensured non-contracture of the excision site. The fistulous connection between the bladder and upper vagina closed spontaneously. The patient has since been having normal menses for the past 15 months with no associated hematuria. The vagina has remained patent since. We conclude that for cases of transverse vaginal septum associated with menouria excision of the septum that ensures normal outflow of menstrual blood is sufficient to facilitate spontaneous closure of the fistulous connection between the bladder and vagina.
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