DOI: 10.36347/sjmcr.2018.v06i05.014

Pages: 340-344

MRKH syndrome is a rare congenital anomaly characterised by uterine and vaginal hypoplasia. Normal external physical appearance precludes diagnosis until puberty with primary amenorrhoea being presenting symptom in majority. The ovarian function and karyotype are normal. MRI is the imaging modality of choice for evaluation of uterus, ovaries and vagina. The syndrome is classified into two types with type I being confined to reproductive system and type II having additional anomalies most frequent being of urinary system. We report a case of 22 year old patient presenting with primary amenorrhoea with absent uterus and right kidney as seen on ultrasonography with MRI confirming the same findings.