Mayer-Rokitansky-Kuster-Hauser syndrome

Sagittal T2FS image showing vaginal and uterine agenesis

Axial image showing normal left ovary

Axial image showing absence of vagina

17 year old patient presenting with primary amenorrhoea. MR reveals uterovaginal agenesis. Ovaries and kidneys were normally present.

Combined uterovaginal agenesis is the most common type of class I mullerian duct anomalies.
In Mayer-Rokitansky-Kuster-Hauser syndrome, patients usually have  agenesis or hypoplasia of upper two-thirds of vagina with intact ovaries. There may be variable anomalies of uterus, urinary tract and vertebrae.