Erdheim-Chester disease in a child with MR imaging showing regression of marrow changes.

Objective: Erdheim-Chester disease is a disseminated xanthogranulomatous infiltrative disease of unknown origin that generally presents in adulthood. A review of the English-language literature demonstrated that pediatric cases were extremely rare, and to our knowledge, only two cases, a 7- and 14-year-old, have been published.

Design And Patient: We report a case of Erdheim-Chester disease in a 10-year-old girl evaluated with MR imaging.