A 20 year old nulligravid, presented with primary amenorrhea, short stature, multiple exostoses and delayed mental development. She complained of cyclic pelvic pain. Her karyotyping showed 46,X,t(X;8)(q24;q22). Pelvic ultrasound done on her showed a diagonal vaginal septum, hematosalpinx and hematotrachelocolpos. On exploratory laparotomy, she also had a unicornuate uterus with hematometra and a non-communicating, non-functioning right rudimentary horn. Hysterectomy was done on this patient. In a review of available literature, this may not be the first case reported to describe occurrence of congenital reproductive tract anomalies in combination with gonadal dysgenesis. However, this is a case with a rare combination of congenital anomalies. Its possible pathogenesis, treatment and implications on the patient's reproductive future are discussed.