In 1947 sidney farber (1) observed an apparently new disease entity in an infant fourteen months of age, which he termed“disseminated lipogranulomatosis.” This, he suggested, might represent a bridge between two apparently etiologi-cally distinct groups of lipid metabolic disorders which he defined as: (a) true metabolic disorders, including Gaucher's disease, Niemann-Pick's disease, and Tay-Sachs' disease and (b) entities not believed to be primarily metabolic but rather inflammatory in the broad sense, and characterized by initially granulomatous lesions which subsequently undergo partial lipid replacement. This latter group includes Hand-Schüller-Christian disease, eosinophilic granuloma, and Letterer-Siwe's disease, all of which are now considered variations of a similar underlying process. Subsequently Farber investigated 2 further examples (2). The 3 cases presented very similar bizarre clinical and pathological findings, which were also noted in the case to be reported here. Case Report G. R. C, a 15-month-old Caucasian male, was admitted to The Johns Hopkins Hospital on May 22, 1957, for diagnostic evaluation. The child was born in a military camp in Japan and was considered to be full-term and normal. The family history, gestation, and neonatal periods were unremarkable. At the age of three months, feeding difficulties, anorexia, projectile vomiting, marked swelling of the wrists, and a peculiar hoarse cry, as well as a low-grade fever, were noted. Treatment with antibiotics and oxygen at the local military hospital resulted in some clinical improvement. Subsequently, marked respiratory difficulty developed and the baby was readmitted to the hospital on several occasions. Bronchoscopy revealed a“fibroma of the left vocal cord.” Because of respiratory distress, a tracheotomy was performed. The child was later transferred to the Walter Reed Army Hospital for an intensive diagnostic study. At that time swellings of the joints of the fingers, hands, elbows, wrists, and knees were noted. Extensive laboratory studies were within normal limits except for an increased creatine output. A therapeutic trial of cortisone was unsuccessful, and a tentative diagnosis of arthrogryposis was made. Physical examination at the time of admission to The Johns Hopkins Hospital revealed a small infant who exhibited marked weakness and lack of voluntary motion. The skin showed a pale yellow color without evidence of frank jaundice. All peripheral joints were grossly deformed by large, knotty, periarticular masses with a rather firm and wooden feeling, as well as by multiple flexion contractures. The joints, however, did not appear to be tender or warm, or to show any signs of inflammation. An extensive firm, knotty mass was also noted in the subcutaneous tissue of the paraspinal area, extending from about D-9 through L-3.