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Dear Editor, Neonatal hemochromatosis (NH) is a severe, progressive, lifethreatening disorder that leads to hepatic failure during the first weeks of life and is associated with massive iron deposits. Although its exact etiology remains to be better clarified, it has been hypothesized that maternal alloimmunity and mitochondrial dysfunction might play important roles in this disease. This condition differs from the main disorders that present with liver iron deposits, such as HFE-related hemochromatosis (autosomal recessive disorder caused by biallelic mutations in HFE gene in which iron deposits lead to adult-onset liver, heart, testicular and pancreatic involvement; OMIM#235200), juvenile hereditary hemochromatosis (autosomal recessive condition associated with biallelic mutations in HJV and HAMP genes and clinically similar to HFE-related hemochromatosis, but earlier onset and fast progression; OMIM#602390 and 613313), TFR2-related hereditary hemochromatosis (autosomal recessive condition associated with biallelic mutations in TFR2 gene and clinically similar to HFE-related hemochromatosis, but earlier onset and slow progression; OMIM#604250) and ferroportin (SLC40A1)-related iron overload (autosomal dominant condition associated with monoallelic mutations in SLC40A1 gene; OMIM#606069). Our male infant was born at term (39 weeks of gestation, birth weight: 2,615 g), after an uneventful pregnancy. His parents were healthy, caucasian, non-consanguineous and have not reported any familial history of liver, genetic, metabolic or syndromic conditions. His general conditions deteriorated after delivery and he required intensive care unit admission for anemia, bleeding, hypoglycemia, hypoactivity and unconjugated hyperbilirubinemia. During the following days, he developed recurrent hypoglycemia, cholestatic jaundice, hepatomegaly and hepatic dysfunction. He was then transferred to our tertiary-care unit at the 33rd day of life. Laboratory tests demonstrated anemia (hemoglobin 9.1 g/dL; hematocrit 26.3%), thrombocytopenia (platelet count: 49,000/μL), abnormal synthetic liver function (International Normalised Ratio Biochemical profile in an infant with neonatal hemochromatosis shows evidence of impairment of mitochondrial long-chain fatty acid oxidation