- 1 in 4000-5000
- second most common cause of primary infertility
- vaginal agenesis or proximal and middle segment hyperplasia
- intact ovaries and fallopian tubes
- varying uterine anomalies – agenesis, hypoplasia, uni or bicornuate
- renal agenesis, pelvic kidney
- normal external genitalia
- amenorrhea
- cyclic pelvic pain