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Hypoglycemia due to 3β-Hydroxysteroid Dehydrogenase type II Deficiency in a Newborn. BACKGROUND: 3β-hydroxysteroid dehydrogenase type II deficiency results in decreased production of all three groups of adrenal steroids. Recurrent hypoglycemia as a presenting feature of this disorder has not been reported earlier. CASE CHARACTERISTICS: A genotypically and phenotypically normal female newborn delivered by in-vitro fertilization presenting with recurrent hypoglycemia. Primary adrenal insufficiency with insignificant mineralocorticoid deficiency and slightly elevated levels of 17-hydro-xyprogesterone, dehydroepian-drosterone sulphate and testosterone. OUTCOME: Successfully managed only with corticosteroid replacement. MESSAGE: Congenital adrenal hyperplasia can rarely cause recurrent hypoglycemia in newborns.