Objective　To explore the diagnosis and treatment of neonatal hemochromatosis (NH). Method　The clinical data of NH in a child were retrospectively analyzed and the related literature was reviewed. Result　A female infant showed no obvious abnormalities in fetal and birth examinations. Skin yellowing occurred 8 hours after birth and aggravated progressively, and no improvement was observed after transfusion and albumin infusion. Liver pathology showed diffuse ballooning of hepatocytes with multinucleated giant hepatocyte transformation, intrahepatic cholestasis with formation of bile duct dilatation and thrombosis, scattered extramedullary hematopoietic foci, and a large number of brownish yellow particles deposited in hepatocytes. Prussian blue staining showed iron deposition, and NH was diagnosed. Subsequently, the patient was treated with N-acetylcysteine and the disease was gradually improved. Conclusion　NH is a severe liver disease with extrahepatic hemosiderin deposition in neonatal period. Iron deposition in hepatocytes by liver pathological examination is the gold standard for diagnosis.