Sturge-Weber syndrome has three main features: cutaneous capillary angiomatosis of the face, choroidal angiomata, and leptomeningeal angiomatosis. The possibility of Sturge-Weber syndrome should be considered in neonates with cutaneous capillary angiomatosis in the distribution of the trigeminal nerve, buphthalmos or glaucoma, or seizures. The diagnosis of Sturge-Weber syndrome should be reserved for neonates with leptomeningeal angiomatosis or, in the absence of leptomeningeal angioma, for neonates with facial cutaneous capillary angiomatosis and choroid angioma. Neonates with facial cutaneous capillary angiomatosis regardless of its distribution, or choroidal angiomas alone, do not have Sturge-Weber syndrome.