A 15-month-old boy was seen because of two distinct types of lesions, namely, yellowish papules on the scalp and face, and hemorrhagic macules and papules on the trunk. A biopsy specimen from one of the yellowish papules showed histopathologic and immunohistochemical changes of both juvenile xanthogranuloma and Langerhans cell histiocytosis. The section from the center of the biopsy specimen showed a proliferation of foamy histiocytes, among them Touton giant cells, which were positive for CD68, but negative for S-100 and CD1a. At the edges of the specimen was a predominantly histiocytic infiltration in the papillary dermis that was positive for S-100 and CD1a, but negative for CD68. The patient died 12 days after hospital admission consequent to disseminated intravascular coagulopathy. We did not biopsy the hemorrhagic lesions; however, this combination of findings suggests a possible relationship between juvenile xanthogranuloma and Langerhans cell histiocytosis, as previously reported.