We report three cases of epidural lipomatosis including one in a patient with primary Cushing's disease. Our literature review found 16 additional cases of symptomatic epidural lipomatosis in patients who were not receiving corticosteroids. The presenting symptoms were nonspecific. The main clinical symptoms were nerve root pain, weakness of the lower limbs upon exertion, paraparesis or isolated back pain. Degenerative lesions were common and were sometimes the cause of the symptoms. Cases were evenly distributed between the thoracic and lumbar spine. Of the 18 patients, 14 were men and eight were older than 54 years. Three-fourths of patients were obese. Spinal cord or nerve root compression occurred in some instances. Modern imaging techniques (computed tomography and magnetic resonance imaging) can establish the diagnosis rapidly. In patients without neurologic compromise, surgery should be considered only if symptoms fail to respond to weight reduction. The rate of occurrence of epidural lipomatosis in patients with Cushing's disease is probably underestimated. Routine investigation by magnetic resonance imaging of Cushing's disease patients who have manifestations known to occur in epidural lipomatosis would allow to evaluate the role of increased production of endogenous corticosteroids in the occurrence of epidural lipomatosis.