We present a sporadic infantile case of primary cerebral mineralization with unexpected asymptomatic clinical course. An 11 months old boy with negative familial and gestational data developed normally. He died after two days of fever and rapid course of cardiorespiratory failure due to pneumonia. Parathyroid dysfunction and somatic abnormalities were not evident clinically. Neuropathological examination revealed bilateral, diffuse or pericapillary calcifications in the striatum, cerebral and cerebellar cortex, dentate nucleus and brain stem. The mineralizations were less prominent in the cerebral hemispheres than in cerebellum. A diffuse demyelination was seen in the cerebellum where calcifications were numerous. We suggest that the intracerebral calcifications progressed gradually through a disease course and probably started in the cerebellum. We discuss the lack of clinico-pathological correlations and the nosologic position of the observed syndrome.