A 61-year-old woman with a 6-year history of chronic myelogenous leukemia (CML) presented with recurrent fever in July 1996. Bone marrow aspiration, biopsy and chromosome analysis showed that CML was in the chronic phase. Bone marrow biopsy revealed nonspecific inflammatory lesions. Chest X-ray and computed tomography examinations demonstrated interstitial pneumonia. Cultures of gastric juice and bone marrow yielded colonies of Mycobacterium avium complex (MAC), and a diagnosis of disseminated atypical mycobacteriosis was made. Multidrug treatment including rifampicin, ethambutol, clarithromycin and ciprofloxacin was begun. The cultures subsequently became negative and the fever was resolved. However, fever eventually recurred and the patient died of multiple organ failure in October 1997. since disseminated atypical mycobacteriosis complicating hematological disorders worsens the prognosis, its early diagnosis and prompt treatment are important. Although it is often difficult to identify atypical mycobacterium as a causal agent, frequent culturing of atypical mycobacterium from various sources including bone marrow fluid can be helpful for early diagnosis whenever fever of undetermined origin occurs in patients with hematological disorders.