OBJECTIVE, PATIENTS, AND METHODS: A severe case of cerebral air embolism after unintentional central venous catheter disconnection was the impetus for a systematic literature review (1975-1998) of the clinical features of 26 patients (including our patient) with cerebral air embolism resulting from central venous catheter complications. RESULTS The jugular vein had been punctured in eight patients and the subclavian vein, in 12 patients. Embolism occurred in four patients during insertion, in 14 patients during unintentional disconnection, and in eight patients after removal and other procedures. The total mortality rate was 23%. Two types of neurologic manifestations may be distinguished: group A (n = 14) presented with encephalopathic features leading to a high mortality rate (36%); and group B (n = 12) presented with focal cerebral lesions resulting in hemiparesis or hemianopia affecting mostly the right hemisphere, with a mortality rate as high as 8%. In 75% of patients, an early computed tomography indicated air bubbles, proving cerebral air embolism. Hyperbaric oxygen therapy was performed in only three patients (12%). A cardiac defect, such as a patent foramen ovale was considered the route of right to left shunting in 6 of 15 patients (40%). More often, a pulmonary shunt was assumed (9 of 15 patients; 60%). For the remainder, data were not available. CONCLUSIONS When caring for critically ill patients needing central venous catheterization, nursing staff and physicians should be aware of this potentially lethal complication.