The authors present the case of a 45-year-old man suffering from progressive quadriplegia due to an expansive C3-T2 epidural mass. Neuropathological examination demonstrated pseudotumor tissue. The patient had had an orbital pseudotumor 5 years before admission, and other systemic manifestations of an idiopathic inflammatory disease were discovered. This case is extremely rare. Nine cases of multifocal fibrosclerosis with central nervous system involvement are described in the literature. To the authors' knowledge, this is the first description of a cervical epidural pseudotumor. Modern imaging has made the diagnosis of such an entity possible, and it is important for the neurosurgeon to consider this syndrome because the combination of surgery and systemic medical therapy can ensure a long-term survival with good quality of life.