A 10-year-old boy with adrenoleukodystrophy was treated with gammaglobulin in conjunction with a mixture of glyceryl trioleate and glyceryl trierucate. With a high dose of gammaglobulin, clinical improvement, including the reduction of visual field defects, was noted. On magnetic resonance imaging, attenuation of the enhancement of the rim with gadolinium was observed, suggesting repair of the blood-brain barrier. When auditory agnosia developed later, a temporary improvement was again obtained with gammaglobulin. Although the progress of the disease could not be arrested permanently, gammaglobulin therapy seemed to have been associated with temporary improvement of the clinical symptoms in this patient with adrenoleukodystrophy.