Two neonates with tuberous sclerosis are reported. The patients presented convulsive seizures 30 mins or 5 days after birth. Computer-assisted cranial tomography (CT) on admission showed typical paraventricular calcifications in Case 1 and a calcified mass in Case 2. In addition to the CT findings, the diagnosis was made on the basis of the depigmented skin, and the mother with tuberous sclerosis in Case 1 and the pathological findings after surgery in Case 2. The importance of CT as a diagnostic procedure for this disease within 1 wk after birth is emphasized.