More than a century after the description of sarcoidosis, the disease remains not well understood. Sarcoidosis is an inflammatory disease of unknown etiology characterized by noncaseating granulomas with multiple organs affected. The epidemiology reveals lung involvement in 90- 95% of the patients and just 5-13% incidence of neurological involvement. We present an unusual case of a patient with medulla oblongata and retroperitoneal sarcoidosis with no other organ involvement. In addition to the case presentation and extensive up-to-date literature review on extrapulmonary sarcoidosis, we describe the difficulties in making the diagnosis and the challenge in differentiating sarcoidosis from other illnesses such as tuberculosis.