The Sheep in the Wolfs Clothing-A Rare Case Report

Isolated Deficiency of Mullerian Inhibiting substance (MIS) is a rare syndrome that usually does not present in the newborn period as the as genitilia appear to be those of a male with undescended testes. Its fascinating as the phenotypic findings are those expected in 46XY. The defect lies in complete failure of the testis to produce MIS in males. This report is of a 35 year old with bilateral undescendedtestis with left hernia diagnosed to be deficiency of MIS.