Abstract

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome, Type 2 is a form of mullerian agenesis typically associated with renal, skeletal, and ovarian abnormalities. It has an incidence of 1 in 1,00,000 in general population. This is an unusual case of a 13-year-old premenarchal girl with severe pain in the right flank. MRI revealed a rare combination of hypoplastic uterus with cervicovaginal agenesis with unilateral ureterorenal and ovarian agenesis. On laparotomy, left ureter was found to be grossly dilated and enveloped by fibrous tissue causing distal ureteric obstruction. Hysterectomy along with ureterolysis, ureteroneocystostomy with psoas hitch was performed. One should be aware of these rare complications associated with MRKH so as to suspect and manage them timely to decrease the morbidity and mortality