Encephalotrigeminal angiomatosis, also called Sturge-Weber syndrome (SWS), is a syndrome of etiology which is not yet clear. It is a nonhereditary condition. The clinical features include pigmentation over the facial skin known as port-wine stain, abnormalities of ocular region, and central nervous system involvement as leptomeningeal angioma. In this manuscript, we present a rare case report with an unusual combination of SWS with facial lobular capillary hemangioma.