Abstract: A 5-year-old boy with Job's syndrome was reported. Major clinical features of the patient included "cold" and recurrent staphylococcal abscesses, recurrent infection of skin and lung, markedly elevated serum IgE values, eczema, "lion" face, hyperextensible joints, absence of patella, slightly elevated eosinophilic cells, normal neutrophil phagocytic activity and normal N. B. T. Histological features of the skin lesion showed gronulomatous inflammatory infiltration. Transmission electron microscopy of the bone marrow showed changes of the neutrophilic cell structure.