Erdheim-Chester disease usually involves the diaphyseal and metaphyseal reg
ions of tubular bones and various visceral organs. A 56-year-old woman pres
ented with the histologically confirmed diagnosis of Erdheim-Chester diseas
e. A Tc-99m MDP bone scan revealed the entire extent of the skeletal diseas
e and showed unusual involvement of the epiphyses and axial skeleton. In ad
dition to MRI, a Ga-67 citrate scan including SPECT showed extensive soft-t
issue infiltration of different organs. Both Tc-99m MDP and Ga-67 scintigra
phy are useful tools in determining the distribution of this rare disease.