Abstract:  Lipodystrophia centrifugalis abdominalis infantilis is a localized lipodystrophic disease affecting young children. A total of 168 cases were collected from dermatology literature. Where possible, details of the data were also obtained from the authors. Most of the patients were Japanese, Korean, or Chinese in origin. The Caucasian patients were from England, Italy, France, Germany, Spain, and the United States. The male-to-female ratio was 1:1.6. The depressed lesions developed during the first 4 years of life in the groin or axilla, surrounded by a slight erythematous change. Histologically, subcutaneous fatty tissue was lost or markedly decreased in the depressed areas, and marked or moderate mononuclear cell infiltration was observed in the fatty tissue of the surrounding erythematous area. The depressed lesions extended centrifugally to involve the abdominal or chest walls. However, in most cases, such enlargement spontaneously ceased by the age of 13 years. No drugs used were effective in preventing the enlargement of depressed lesions, but more than 60% of patients showed spontaneous improvement after the cessation of enlargement. Lipodystrophia centrifugalis abdominalis infantilis is a clinically and histologically distinct lipodystrophic disease affecting young children, mostly in Asian countries, but a few in Western countries.