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Erdheim-Chester disease (ECD) is a systemic histiocytic disorder that occurs mainly in adults and is rarely found in children. ECD is characterized by bilateral, symmetric osteosclerosis of the metadiaphyses of the long tubular bones. We report a 10-year-old girl with ECD who presented with pain of both knees and elbows that involved the long bones of the upper and lower extremities, the metacarpals, and facial bones. Tc-99m MDP bone and Ga-67 citrate scintigraphy showed the typical pattern of symmetric involvement of the long bones and the facial bones, and was useful in diagnosis and determining the extent of ECD.