INTRODUCTION: Herlyn-werner-wunderlich syndrome is a rare congenital malformation of the mullerian ducts. It is stipulated to be a developmental abnormality of the wolffian ducts as well as of that of the mullerian ducts. Also known today as uterine didelphys associated with obstructed hemivagina and ipsilateral renal anomaly (ohvira), a broader term, to include other types of renal abnormalities.Significant occurrence is noted commonly on the right side and there is no clear explanation for this fact. The disease is rare and prevalence is undetermined. In the general population, unilateral renal agenesis is estimated to be from 1 in every 600-1200 individuals. In women, the prevalence of genital abno rmalities associated with kidney anomalies is estimated between 25-89%. The abnormalities of the urinary tract most frequently associated with developmental abnormalities of the mullerian ducts are renal agenesis, double collecting system, double kidney and horseshoe kidney.