SINCE Hurler's1description of 2 cases in 1919, a syndrome consisting of enlarged, abnormally shaped head, dwarfishness, grotesque facies with sunken nasal bridge, cloudiness of the corneas, hepatosplenomegaly, umbilical and inguinal hernias, dorsolumbar kyphosis and flexion deformities of the extremities has been recognized. It has been known, under various names, as gargoylism, Hurler's syndrome, Hunter's syndrome, lipochondrodystrophy, osteochondrodystrophy and dysostosis multiplex. Although it is often referred to as Hurler's syndrome, Hunter2two years prior to the publication of Hurler's paper described the condition in two brothers. To date, approximately 100 cases of lipochondrodystrophy have been described in the literature. However, in this large number of reports there have been only 14 comprehensive general pathologic studies and 8 analyses of the changes in the nervous system. On such meager studies, the prevailing opinion places lipochrondrodystrophy in the group of lipid storage diseases related to amaurotic familial idiocy and to