Cerebellitis is not a recognised manifestation of Lyme disease. We describe a patient with clinical features of subacute cerebellitis, cerebrospinal fluid (CSF) monocytic pleocytosis, positive CSF Borrelia burgdorferi antibodies, negative brain magnetic resonance imaging and a benign course after treatment with ceftriaxone. Possible earlier cases are discussed. Lyme disease should be considered in all cases of subacute cerebellitis.