A non-caseating granuloma is the histological hallmark of sarcoidosis, a multisystem disease. Neurosarcoidosis is a complex and rare form of sarcoidosis that affects the CNS . It involves the spinal cord with intradural lesions. Herein, we present a case of neurosarcoidosis with a family history of MS . He first underwent surgery for swelling of the right wrist joint and then was diagnosed with multiple sclerosis due to the onset of paraparesis and Magnetic resonance imaging (MRI) results. After an open biopsy, the diagnosis of neurosarcoidosis was established and was followed up by appropriate medical management. In conclusion, to distinguish MS from neurosarcoidosis, CSF analysis may not be particularly useful since it may reveal similar abnormalities. All manifestations are not identical between MS and neurosarcoidosis; nonetheless, persistent meninges enhancements or parenchymal enhancements in tissue are not expected in MS and likely indicate a granulomatous process. The administration of appropriate treatment at an early stage of the disease can assist in decelerating its progression.