We report an autopsy case of congenital cytomegalic inclusion disease that was associated with massive periventricular calcification and hydrocephalus. The male boy was bron by cesarian section at 38 weeks of gestational age to a 28 year old mother and was dead two hours after delivery because of respiratory difficulty. Radiologic findings showed characteristic linear periventricular calcification, being associated with marked dilatation of the lateral ventricles. At autopsy, the brain parenchyme became 0.4 cm in average thickness due to obstructive hydrocephalus at aqueductal level. The inner surface of the lateral ventricle showed grayish white granular appearance that could correspond to the calcification seen on plain skull X-ray films. Although active ependymitis, destruction of the brain parenchyma with dystrophic calcification and gliosis were noted, there was no intranuclear inclusion. After scrutinizing many sections of various organs and tissue, we could be able to demonstrate a few inclusion bodies, highly suggestive of cytomegalic inclusion disease. It was discussed that this case might suggest that intranuclear inclusions in cytomegalovirus infection could by very difficult to find particularly when the infection is in inactive phase despite the presence of tissue necrosis and calcification of the brain.