Mullerian anomalies are a relatively uncommon occurrence with implication for adolescents and adults as they may result in specific gynaecologic, fertility and obstetrical issues. Uterus didelphys with blind hemivagina and ipsilateral renal agenesis is a rare congenital anomaly. Patient may be asymptomatic and unaware of having double uterus or may present with severe dysmenorrhoea or dyspareunia or a palpable mass due to unilateral haematocolpos. We report a case of 12 year old girl with this condition who was diagnosed as uterus didelphys with unilateral haematocolpos with ipsilateral renal agenesis on the basis of clinical association, physical examination and sonography and intravenous urogram.