Sarcoidosis is a multisystem disease of unknown etiology, characterized by the presence of noncaseating epithelioid granulomas and accumulation of T lymphocytes and mononuclear phagocytes, which damages the normal structure of tissues. Isolated form of neurosarcoidosis is very rare and difficult to diagnose and requires histologic confirmation of noncaseating granulomas in the nervous tissue. We report a case of a 55-year-old female who had probable isolated neurosarcoidosis based on magnetic resonance imaging findings of relapsing pachymeningitis with an inflammatory process in the apex of the right orbit and pseudotumor inflammation of the superior and lateral recti of the right eye. Diagnosis was further verified by positive response to dual corticosteroid and immunosuppressive therapy. Our case demonstrates the importance of considering isolated neurosarcoidosis as a potential underlying etiology of painful ophthalmoplegia, even without systemic manifestation of the disease.