We report a Japanese girl with atypical lipodystrophia centrifugalis abdominalis infantilis. The initial lesion developed on the neck as an area of erythema which showed centrifugal spread to the nape, submandibular area and upper breast and central fading to leave a residual depression and purplish brown pigmentation symmetrically. A central depression on the neck, nape, submandibular area and upper breast was surrounded by a distinctive erythematous, slightly elevated and indurated border. Histological examination of the erythematous border revealed inflammatory changes in the subcutaneous fat. Although this patient was affected in an unusual site, we concluded that she had lipodystrophia centrifugalis abdominalis infantilis, because of the overall features of the lesions.