Persistent Muellerian duct syndrome is a very rare anomaly. A woman of 46-year-old was admitted with the history of lower abdominal pain, bleeding per vaginum and irregular menstrual cycles for last 6 months. An irregular non-tender mass of 16 x 10cm in size was palpable in suprapubic region. Per vaginal examination revealed two cervices and an incomplete vaginal septum. Ultrasound abdomen showed one uterus with endometrial cavity clearly delineated with a fibroid and an ovarian cyst in the left lumbar region. On laparotomy, 2 uterii along with a fibroid from the anterior wall of left uterus and a left ovarian cyst was seen. Patient had undergone total abdominal hysterectomy and adnexa removal. The specimen showed florid adenomyosis with leiomyoma of fibroid.