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Juvenile xanthogranuloma (JXG) is a benign tumor of the skin that occurs most often on the head and neck of infants and young children. This growth usually goes away without medical intervention. The tumor consists of an accumulation of histiocytes and Touton giant cells in the dermis, or the inner skin layer. Histiocytes are cells that are present in loose connective tissue, and Touton giant cells are large cells with many nuclei, often found in xanthomatosis, a condition characterized by the deposition of fats in tissues. Cases are described of 17 patients with a variant form of JXG. These cases of variant JXG had unusual tissue microscopic features, were difficult to diagnose, and were sometimes confused with malignancies. The clinical characteristics and microscopic features of this variant of JXG are described. A peculiar feature observed was the absence of Touton giant cells and foam cells, which contain a foamy cytoplasm. In addition, the number of dividing cells was large. (Consumer Summary produced by Reliance Medical Information, Inc.)