Sarcoidosis is a rare systemic disease characterized by growth and organization of inflammatory cells in a granuloma. Granulomas can localize in any parts of the human body. The main localization is represented by lungs, lymph nodes, eyes and skin. Any organ, however, can be affected. Central nervous system (CNS) represents a rare localization of sarcoidosis, in fact, only 1% of patient with sarcoidosis present brain and/or spinal cord localization of the granulomas associated with this disease. This condition takes the name of Neurosarcoidosis. Its diagnosis and management pose a significant challenge, as its clinical manifestation and appearance in imaging studies are difficult to distinguish from more common lesion of the spinal cord. In this paper, we present the case of a 45-year-old woman who presented back pain associated with neurologic signs of myelopathy. Spinal cord MRI documented a T2 hyperintense signal around medullary conus. She underwent a gross total removal of the lesion. Histopathological examination revealed spinal sarcoidosis. Next total body CT scan did not show other localization of the disease. The aim of the present paper is to report a very rare case of spinal epidural sarcoidosis, actually only five cases have been described, without other localization of the disease. The present article underlines the difference between this forms of spinal sarcoidosis compared to intradural extramedullary and intramedullary spinal sarcoidosis.