- Neonatal hemochromatosis (NH) is a rare disease which has been characterized as severe neonatal liver disease in association with extrahepatic siderosis in a distribution similar to that seen in HFE-associated hereditary hemochromatosis. The clinical presentation is severe liver dysfunction that could lead to intrauterine fetal death or the neonate death. The treatment is antioxidants, chelation or cocktail liver transplant. The prognosis is very bad with less than 40% survival. The etiology and pathogenesis are yet unknown. It has been considered to be a syndrome in which a number of primary etiologies, such as infection, genetic-metabolic disease and toxic insult, lead to a common phenotype. The common hypothesis now is that NH is a consequence of gestational alloimmune disease. The basis for this theory rests on the pattern of recurrence. Little direct evidence for an immune mechanism exists. The fact that high-dose intravenous immunoglobulin appears to lessen the severity, improve infant survival, but not prevent its recurrences, strengthens this idea. The authors present a twenty nine years old Bedouin woman, married with no children. Her bad obstetrical history included one first trimester spontaneous abortion, five intrauterine fetal death and one post partum death. The present review discusses the clinical and epidemiological characteristics of this disease, and focuses on the etiology and treatment.