Abstract: ObjectiveTo study the clinical features of gestational alloimmune liver disease-neonatal hemochromatosis(GALD-NH), and to improve the understanding of GALD-NH.MethodsClinical manifestations and pathological diagnosis process of one patient with GALD-NH were retrospectively analyzed. The GALD-NH related literature was reviewed.ResultsThe patient was a full-term male infant. Onset of the disease was the second day after birth. Clinical characteristics included severe liver function dysfunction, coagulation function dysfunction, oliguria, anasrca (hydrothorax and ascites), thrombocytopenia. The patient died on 43rd day after birth. Pathological anatomy showed severe acute liver failure. Pathologic siderosis of extrahepatic tissues was positive, incluing pancreas and thyroid gland. Liver biopsy showed strong immunostaining of hepatocytes for the C5b-9 complex. Definite diagonosis of GALD-NH was made for this patient. Totally 90 patients with NH were recruited from 12 articles and our report. Main prenatal signs were hydrops fetalis(17/90), oligohydramnion(21/90), intrauterine growth retardation(33/90), and clinical process after birth developed rapidly with abnormality of iron metabolism(65/90), liver function failure(62/90) and severe coagulation function dysfunction(58/90). Extrahepatic siderosis and positive C5b-9 complex of hepatic tissue were confirmed in 48 and 14 patients respectively. The number of patients received Cocktail treatment, IVIG transfusion, blood exchange transfusion(BET), IVIG+BET and liver transplantation were 36, 4, 9, 20 and 13, respectively. Fifty-five patients died and 35 survived. Twenty-eight of survivors were followed and the prognosis was good. ConclusionGALD-NH is rare and fatal, processing quickly after birth in multisystem injuries involving iron metabolism, liver and coagulation. Diagnosis of NH needs presence of extrahepatic siderosis. Treatment strategy includes IVIG infusion and/or BET. Mortality of GALD-NH is high, but prognosis is good for survivor.