A milder, ‘non-classic’ form of lipoid CAH without phenotypic sex reversal in 46,XY patients was reported ten years later (Baker et al. 2006) and has now been confirmed in multiple reports (Sahakitrungruang et al. 2010, Altinkilic et al. 2024, Phadte et al. 2024), greatly expanding the clinical situations in which StAR mutations must be considered. The gene discussed is STAR; the disease is congenital adrenal hyperplasia.