Granulomatous disease of childhood is a chronic and eventually fatal, genetically transmitted syndrome usually affecting male children. Recent investigations have shown that the polymorphonuclear leukocytes and monocytes of these patients phagocytize normally but are unable to kill certain organisms. As a result, these children manifest a granulomatous response in many organs. Typically they develop eczematoid eruption, lymphadenitis with suppuration, hepatosplenomegaly, and repeated, slowly resolving pneumonias. Eight of twenty-eight patients developed osteomyelitis with distinctive clinical and roentgenographic findings. Thus, clinical, general roentgenographic, and orthopaedic manifestations are typical of the disease. Definite diagnosis is readily established by one or more relatively simple laboratory studies. Because of the ease of diagnosis and the importance of recognizing chronic granulomatous disease, the orthopaedist should be aware of its manifestations.