The syndrome of associated facial hemangioma and intracranial lesion is characterized by the presence of two outstanding signs: (1) a facial port wine nevus (fig. 1) present at birth, which follows the distribution of one or more branches of the fifth cranial nerve, and (2) calcification of the occipital lobe of the cerebrum on the same side as the nevus. This disease has been called after a number of the investigators who have reported cases, including Kalischer,1 Weber,2 Krabbe and Dimitri, each of whom was cited by Ford,3 Brushfield and Wyatt4 and Sturge, cited by Newson.5 It has also been referred to as cerebral telangiectasis and, more recently, as associated facial and intracranial hemangioma.
Kalischer was the first to describe the syndrome, in 1897. In 1906 Cushing6 stated that facial nevi show a tendency to conform in outline to the distribution of one or