Idiopathic chronic neutropenia is characterized by a variable pattern of age at the onset of neutropenia, neutrophil counts, maturational arrest at the promyelocyte/myelocyte stage in the bone marrow, and recurrent pyogenic infections without any underlying disease or drug administration. There have been reports of lack of response to conventional-dose steroid therapy, but in recent studies it has been shown that high-dose corticosteroids are effective in several neutropenic states. Here we report an apparent response to high-dose methylprednisolone in a patient with idiopathic chronic neutropenia.