Uterine didelphys is a congenital malformation characterized by the presence of two separated hemiuteri and hemivaginas, due to lack of midfusion of the Mullerian ducts. We report a case of UD-BHRA(uterine didelphys with blind hemivagina and ipsilateral renal agenesis), a rare type of uterine didelphys charaterized by symptomatic unilateral hematocolpos due to blind hemivagina after menarche and ipsilateral renal agenesis. The MRI findings in 22-year-old woman with bilateral ovarian tumors demonstrated two separated uterine horns and cervical and vaginal canals, with left hematocolpos and left renal agenesis.