There are very few reported cases of Erdheim-Chester disease that document involvement of dura at the level of the spinal cord. Among these reports, we know of no publication that includes detailed MRI findings. To the best of our knowledge, the case presented here is the first published report of this specific manifestation of Erdheim-Chester disease that includes detailed MRI findings in addition to the related history. Spinal manifestations of Erdheim-Chester disease in our patient were at the dorsal and lumbar levels (T1-T6 and T12-T11 respectively). Both epidural and subdural linear large masses were present, causing spinal cord compression at the dorsal level and epidural thickening at the lumbar level.