Nonclassic congenital lipoid adrenal hyperplasia (NCLAH) was first reported in 2006 due to biallelic pathogenic missense variants in STAR.59 Two brothers had predominant glucocorticoid insufficiency and normal genitalia (p.R188C), and a 46,XX girl had low cortisol and extremely high ACTH (p.V187M) (Figure 3A). Here, STAR is linked to congenital lipoid adrenal hyperplasia due to STAR deficency.