Congenital ichthyosiform erythroderma is a rare dermatosis characterized by erythroderma and hyperkeratosis, more or less generalized, occurring at, or shortly after birth. Brocq1 first described it in 1902. MacKee and Rosen2 reviewed the subject thoroughly in 1917, not only from the historical but also from the clinical and histologic points of view. Since then, there have been several other reports on this condition.3-12 Most of the reports are those of isolated cases, and most authors feel that heredity and sex do not play an important part in the occurrence of this disease. However, there are some cases of familial incidence of congenital ichthyosiform erythroderma. This is most completely reviewed by Touraine4 who states that while the majority of the familial cases show a simple recessive type of inheritance, there is an occasional instance of dominant inheritance. Dr. Samuel Irgang,14 in his long experience, was not