Most cases of double uterus with obstructed hemivagina and ipsilateral renal agenesis were diagnosed at adolescents after menarche. This is the first reported case of complete septate uterus with obstructed hemivagina and ipsilateral renal agenesis in addition to ipsilateral agenesis of fallopian tube and ovary in which the diagnosis was delayed until pregnancy. The pregnancy was uneventful in spite of intermittent vaginal spotting. During the cesarean section, the septum of the uterus was resected and about a 3-cmx3-cm window was made on the vaginal septum to allow an opening for the obstructed vaginal discharge. We followed the patient up for one and half years, and she has not had symptoms such as dysmenorrhea or abnormal vaginal bleeding.