The etiology of sarcoidosis is unknown, but it has long been suspected to be mycobacterial. In the present study, we used 4 mycobacterial species-specific polymerase chain reaction assays on cerebrospinal fluid obtained from a patient with neurosarcoidosis. Positive hybridization was observed with both the Mycobacterium avium complex probe and the insertion element IS900-specific probe that has been found in M. paratuberculosis species. There was no hybridization with M. tuberculosis or M. avium woodpigeon strain-specific probes. This case report demonstrates that M. paratuberculosis or some closely related M. avium spp which perhaps also carry IS900, or contain closely related DNA sequences, are associated with at least some cases of sarcoidosis disease.