Erdheim-Chester disease (ECD) is a rare multisystemic disease characterized by the infiltration of multiple organs by foamy CD68 + CD1a- histiocytes (resident mononucleate macrophages) and “Touton” cells (multinucleated giant cells), which are typical of lesions with high lipid content, such as fat necrosis, xanthomas, and xanthelasmas. Here, CD68 is linked to glycogen storage disease VI.