A patient with Sturge-Weber syndrome without the characteristic facial nevus presented with focal seizures which were difficult to control and borderline mental level. CT disclosed calcification in the right occipital zone. A marked decrease of the regional cerebral blood flow that extended beyond the abnormalities depicted on CT was seen by SPECT. Venous magnetic resonance (MR) angiography revealed reduction of the superficial cortical veins and prominent deep collateral venous system in the same side of the cerebral lesion. Cranial MR imaging with Gd-DTPA demonstrated the pial angioma.