Bilateral undescended testes (BUDT)/inguinal hernias were the presenting features in 16 of 121 (13.2%) children, of whom 5 had CAIS (46, XY), 1 had 17β-hydroxysteroid dehydrogenase deficiency type 3 (HSD17B3) (46,XY), 1 had a WT1-associated condition (46,XY), 5 had persistent müllerian duct syndrome (PMDS) (46,XY), and 4 had MRKH syndrome (46,XX, inguinal hernias). The gene discussed is HSD17B3; the disease is persistent Mullerian duct syndrome.