Over the past 50 years, there have been numerous reports of the triad of blepharoptosis, and epicanthus inversus. The consistency of the triad and its hereditary background make it a true clinical syndrome, and yet it has escaped both colla tion and appellation over the years. This paper presents a collection and com parison of all cases previously reported. We think these illustrate that a fourth finding, telecanthus, is so consistent that it should be added to the triad. A family with this syn drome and several associated features not previously described is also reported. In 1921, Komoto described the first case with the triad, a patient who had, in addition, wide set eyes, ectropion, excessive brow hair, and hypoplasia of the caruncle and plica semilunaris. Three unspecified relatives of this patient were reported to have the same disorder. Later that same year, Dimitry reported a family with blepharophimosis. He did not specify other findings, but from the photo graphs accompanying his report, it appears that this family had the complete triad. Dim itry did, however, trace the pedigree of the blepharophimosis, which is the first and longest (after revision by Owens in 1960) pedigree of this syndrome (Fig. 1). In this pedigree, the syndrome appears in both sexes and is transmitted as a Mendelian dominant characteristic. This mode of inher itance is now well documented. Since these early descriptions, 125 addi tional cases were reported. Of these, 12 were male, six were female, and in 107, sex was