Increased intracranial pressure developed as a terminal event in a 5-year-old boy with adrenoleukodystrophy. The CSF protein concentration was 420 mg/dL. Computed tomography showed extensive areas of decreased density in the posterior cerebral white matter. At autopsy, brain weight exceeded the expected value by 20%, and white matter water content was 87.6% (expected value, 72.5%). In rare instances, brain edema may contribute to the development of increased intracranial pressure in adrenoleukodystrophy.