Two cases of Erdheim-Chester disease are presented: a 26-year-old white male patient with lipoidgranulomatosis of numerous long and flat bones and infiltration of pericardium, pleura, liver, spleen, thyroid, skin, conjunctiva, gingiva, and false vocal cord; and a 54-year-old white male with involvement of bones, orbits, brain, pericardium, and retroperitoneum. The scintigraphic findings in this disease are described, and a comprehensive review of the 27 previously reported cases is given including an assessment of the value of scintigraphy for diagnosis and follow up of this rare disease.