Autopsy findings in an 8 year-old boy suffering from muscular weakness, hypotonia, and psychomotor retardation are presented, who displayed the following morphologic manifestations of infantile neuroaxonal dystrophy (INAD). 1. the presence of numerous spheroids in nearly all parts of the central nervous system, 2. cerebellar atrophy with total loss of PURKINJE and granular cells, 3. optic nerve atrophy, 4. selective distribution of fat-laden cells in globus pallidus, striatum, and substantia perforata anterior, 5. demy-elination of the pyramidal, spinocerebellar and GOLL's tracts. Ultrastructurally the spheroids were filled with cluster of involved mitochondria, osmophilic amorphous materials, increased neurofilaments, abnormal membrano-tubular structures and crystalline-like clefts.