True hermaphrodite presenting as primary amenorrhea.

True hermaphrodite is one of the rare variety of disorders of sex development. Most of them are genotypically females (46 XX) and present as under virilized males. Features of hyperandrogenism are present in those reared as females. The commonest histological variety of gonad found in them is ovotestis, which is at risk for malignancy. We report a girl with 46 XY true hermaphrodite without ovotestis presenting as primary amenorrhea and isolated clitoromegaly in the absence of any other features of virilization.