A rare case of neonatal cholestasis

A term appropriate for gestational age neonate presented with severe cholestasis with features of acute hepatic failure in early neonatal life. There was hypoglycemia, hyperammonemia, cholestasis, ascites, and hepatomegaly. Buccal mucosal biopsy containing minor salivary gland revealed iron deposition on Prussian blue staining suggestive of neonatal hemochromatosis. The neonate responded to exchange transfusion and intravenous immunoglobulin.