Ehrlichia canis infection was diagnosed in a dog with a history of seizures and nonregenerative anemia. Serologic titer to E canis was greater than 1:100. Evaluation of CSF revealed a high cell count, high protein concentration, and a positive Pandy test result. Several mononuclear leukocytes in the CSF contained E canis morulae. Central nervous system lesions are commonly found on postmortem examination of animals with ehrlichiosis, although clinical reports of neurologic signs attributable to this disease are less common. Ehrlichiosis should be included in the differential diagnosis of CNS disease in dogs from enzootic areas.