In 1947, Farber described a syndrome characterized by dysphonia, laryngeal stridor, nodular tumefactions, hyperesthesia and rigidity of the peripheral joints, projectile vomiting, and cutaneous pigmented lesions of the bony prominences (1). Subsequently he reported 2 other cases, describing the entity as “disseminated lipogranulomatosis,” considering it to have some clinical and pathologic similarities to the dyslipidoses (1, 2). Two further cases have been recorded (3–5). All the patients died in infancy. In a similar case reported below, the progression of the radiographic lesions is described, with a pathologic correlation at autopsy. Case Report D. C., a 3-month-old Mexican-American male infant, was admitted to the hospital in May 1962 for investigation of an illness which resembled arthrogryposis multiplex congenita. He was the third child of healthy parents, born at term after an uneventful pregnancy with a weight of 8 lb. 5 oz. During the first months of life, the mother noted tenderness and pigmentary changes over the infant's wrists, elbows, knees, and ankles, with decreased voluntary movement and rigidity of these joints. Physical Examination: Examination revealed a quiet, lethargic infant weighing 11 lb. 9 oz. Bilateral flexion contractures were present in the knees and elbows, and extension contractures in the wrists. Mobility was markedly limited in all major joints, and passive movement of the extremities elicited pain. The muscles of the extremities appeared atrophic but not indurated. Facial asymmetry with occipital and parietal flattening appeared to be caused by the infant's habitual attitude of lying with his face rigidly rotated to the right. Deeply pigmented, velvety macular lesions were present over the bony prominences of the interphalangeal joints, wrists, elbows, knees, and ankles. There was moderate flaring of the lower thoracic cage with each inspiration. Laboratory Investigation: A persistently elevated leukocyte count between 12,000 and 15,000 per cu. mm. and electromyographic changes suggestive of lower neuron involvement were notable. While the patient was in the hospital, profuse watery diarrhea developed; bacteriologic cultures were negative for pathogenic organisms. Subsequent Course: During the next six months, prominent, noninflammatory, periarticular swellings appeared over the metacarpophalangeal joints, wrists, knees, and ankles. A diagnosis of disseminated lipogranulomatosis was suggested by the Dermatology Department, and the infant was readmitted to the hospital in November at nine months of age. Biopsy of a nodular area of the subcutaneous tissue of the ankle confirmed the diagnosis of disseminated lipogranulomatosis. In the ensuing months, the patient experienced recurrent episodes of diarrhea, vomiting, and dehydration.