A rare case of Sturge-Weber syndrome without facial nevus and epileptic seizures is reported. The other cases of incomplete form of the disease reported in the literature showed occipital calcification and epileptic seizures without facial nevus, while in the present case also the convulsions were absent. The possible pathogenic mechanism is discussed. The CT findings of these incomplete forms include unilateral or often bilateral occipital calcifications with no evidence of contrast enhancement. The Authors conclude that the radiologic finding of bilateral gyriform calcifications in the occipital region must suggest the diagnosis of Sturge-Weber disease even in the absence of facial nevus and epileptic seizures.