Introduction The literature comprises 21 reports on cases showing very similar symptom complexes, sufficiently identical to be regarded as a well-defined clinical entity and as a new, autonomous syndrome to be linked into the long chain of heredity of ectodermal dysplasia. The cases which I culled from the literature were described under the following variety of names: sutural congenital alopecia (Aubry, 1893); congenital cataract with cranial anomaly (Bergmeister, 1911); progeria complicated by microphthalmia (Schondel, 1943); progeria with nanism and congenital cataract (Moehlig, 1946): bird face and congenital cataract (Hallermann, 1948); mandibulofacial malformation and ocular changes (Streiff, 1950; Nizetic, 1954); Vogt-Koyanagi-like syndrome with mandibulofacial dysostosis (Ludwig and Korting, 1950); dyscephalia with congenital cataract and hypotrichosis (Ullrich and Fremerey-Dohna, 1953; Weyers, 1954: Leffertstra, 1956); congenital ectodermal dysplasia (Gregory, 1955); progeria with ocular anomalies (Gregersen, 1956); mandibulofacial dysostosis (Blodi, 1957). Positive Signs As I analyzed these cases I found that they invariably showed