Mycobacterium avium complex infections rarely involve the central nervous system, even in patients with widely disseminated disease. We report a case of disseminated M. avium complex infection presenting with central nervous system symptoms in a patient with Hodgkin's disease. Neurologic findings included disorientation, nuchal rigidity, seizures, an extensor plantar response, cerebrospinal fluid studies consistent with meningitis, and eventually, coma and death. Organisms of the M. avium complex were cultured from several sites. Histopathologically, the central nervous system lesions consisted of small aggregates of lymphocytes and macrophages containing acid-fast organisms present in a predominantly perivascular location. In view of the life-threatening nature and potential treatability of this uncommon presentation of M. avium complex infection, we suggest that these organisms be sought in immunocompromised patients presenting with unexplained central nervous system symptoms.