The clinical differentials of sex reversal with adrenal insufficiency include P450scc deficiency due to mutations in CYP11A1 gene, Smith-Lemli-Opitz syndrome (DHCR7 gene), NR5A1 (SF1) mutation-related sex reversal, CYP17 and 3-beta hydroxysteroid dehydrogenase deficiency (HSD3B2 gene) (3). Here, CYP11A1 is linked to Congenital adrenal insufficiency with 46, XY sex reversal OR 46,XY disorder of sex development-adrenal insufficiency due to CYP11A1 deficiency.