A case of degeneration of the gray matter in infancy is reported. An 8 years old boy has repeated generalized seizures, without mental deterioration for over a year. During 6 days he presented diffuse motor deficit and profound coma, till death. Histologically there were found lesions similars to those previously reported as Alpers' disease, i.e. diffuse neuronal loss in encephalic and spinal cord gray matter and disgenetic lesions in cerebral white matter.