The cases of three patients with an atrophic lipophagic granulomatous lipoatrophy are described and compared with 14 similar cases in the literature. In affected children erythematous lesions of the arms and legs usually occur, after which subcutaneous atrophy develops. Fever and edema are common. The histologic feature is a replacement of the fat lobule by lipophagic histiocytes and giant cells. No vasculitis or phlebitis is present. Previously, such cases have been called Weber-Christian disease or Rothmann-Makai syndrome, but they are best characterized by the distinctive clinical lesions, the course, and histologic findings. Corticosteroids apparently have been effective in some patients.