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Glucose 6-phosphate-isomerase deficiency (GPI) is an uncommon autosomal recessive genetic disorder characterized by chronic asphoric hemolytic anemia, jaundice, and hepatospleenomegaly of varying degrees. Avascular necrosis of the femoral head in children may be caused by Legg-calve-perthes disease or hematological diseases. However, to date, there is no literature reporting on femoral head avascular necrosis as a complication of GPI.