The differential diagnoses of our case included progressive familial intrahepatic cholestasis (PFIC), childhood primary sclerosing cholangitis (PSC), congenital hepatic fibrosis, childhood autoimmune hepatitis, childhood primary biliary cirrhosis, alpha-1-antitrypsin deficiency, and cystic fibrosis. This evidence concerns the gene SERPINA1 and hyperinsulinemic hypoglycemia, familial, 4.