Central nervous system (CNS) strongyloidiasis is a known but rare form of disseminated infection. The diagnosis is often made postmortem, with only five published cases of an antemortem diagnosis. We report two fatal cases of CNS strongyloidiasis diagnosed antemortem, with Strongyloides stercoralis larvae visualized in the CNS sample in one case. Risk factors for disseminated strongyloidiasis common to both cases included origination from the Caribbean, underlying human T-lymphotropic virus-1 infection, and recent prednisone use. Both cases occurred in Canada, where the occurrence of Strongyloides is uncommon, and serve as a reminder to maintain a high index of suspicion in patients with epidemiologic or clinical risk factors for dissemination.