17β-Hydroxysteroid dehydrogenase III deficiency
17β-Hydroxysteroid dehydrogenase III deficiency is a rare autosomal recessive disorder of sexual development condition that is a cause of 46,XY disorder of sex development (46,XY DSD). The impaired testosterone biosynthesis by 17β-hydroxysteroid dehydrogenase III (17β-HSD III), presents as atypical genitalia in affected males.
Source: Wikipedia — 17β-Hydroxysteroid dehydrogenase III deficiency (CC BY-SA 4.0)