This is also the first report of classic, severe P450scc deficiency caused by a pathogenic variant in the CYP11A1 gene in a newborn in Poland. The gene discussed is CYP11A1; the disease is Congenital adrenal insufficiency with 46, XY sex reversal OR 46,XY disorder of sex development-adrenal insufficiency due to CYP11A1 deficiency.