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The distinctive features of this case of encephalofacial angioinatosisthe bilateral intracranial calcifications denionstratcd radiologically at birth and the early death of the child with angioniatosis of the skull, leptoineningeal melanosis and cerebral and cerebellar malforinations found at autopsy-extend present knowledge of this entity. An extentive search of the literature on the Sturge-Weber syndrome prompted by this profoundly involved case also led to the conclusion that a detailed review of those contributions which defined this syndrome, with citation of the original sources, was in order. Case R e p o r t : J. H. H., a Caucasian male, horn on August 27, 1962 at Michael ReeseHospital, Chicago, exhibited wide-spread portwine staining of the scalp, face, trunk and extremities (Figures 1 a and b). Birth weight was 4.2 Kg ( 9 lbs. 4 0 2 . ) ; the head circumference measured 35.5 cm. Left-sided buphthalmos and mild glaucoma were observed, for which the infant was placed on acetazoleamide and pilocarpine eye-drops.' General physical examination was reported to he otherwise unremark-