Obstructed hemi-vagina and ipsilateral renal agenesis (OHVIRA) syndrome is a complex of Müllerian and Wolffian duct anomalies constituted by obstructed hemi-vagina that may form hematocolpos with ipsilateral renal anomaly and different degrees of didelphic uterine anomalies. Due to the obstructive anomalies of the reproductive tract, endometriosis of differing severities may be a common complication of OHVIRA syndrome. Here, we report three women with OHVIRA syndrome that showed different clinical courses and severities of pelvic endometriosis. This may suggest a relationship between the pathogenesis of endometriosis and asymmetrical obstructive Müllerian anomalies.
