Iron is a micronutrient which is essential for the existence of organisms. This element participates in oxygen transport, as well as energetic, metabolic and immunologiocal processes. Disturbances of iron homeostasis lead to multiorgan dysfunction. Iron deficiency anemia is a common disorder in childhood, while iron overload is rarely observed in the developmental age. There are primary and secondary reasons for iron overload. Hereditary hemochromatosis is a metabolic disorder caused by the mutations of genes that control iron metabolism leading to increased intestinal absorption. Secondary hemochromatosis is caused by multiple transfusions, chronic hemolysis, or iron pills and iron-rich food intake. The article reviews the literature devoted to primary iron overload in childhood.