Histoplasmosis of local origin has not been reported in humans or wildlife in Alaska, and the disease has never been reported in a free-ranging marine mammal. In 2005 a northern sea otter (Enhydra lutris kenyoni) was found on Kodiak Island, Alaska, at 57° latitude north, far outside the known distribution of Histoplasma capsulatum. The animal died of disseminated histoplasmosis. Microorganisms consistent with Histoplasma sp. were observed on histopathology, and H. capsulatum was identified by PCR and sequencing. We suggest migratory seabirds or aerosol transmission through prevailing winds may have resulted in transmission to the sea otter.