A 6-year-old boy developed hemophagocytic syndrome during the recurrent course of Kawasaki disease. Despite the appropriate treatment modalities for Kawasaki disease, he developed pancytopenia, marked hepatosplenomegaly, high-grade fever, hyperferritinemia, hypertriglyceremia, and evidence of hemophagocytosis in the liver biopsy. Although the course was stormy, he responded well to a combination therapy of corticosteroids, etoposide VP16, and granulocyte colony-stimulating factor G-CSF. The clinical course and the treatment given were compared with the previous reported cases.