Lithium is a neurotoxin with a particular affinity for the cerebellum. The risk of permanent neurotoxic sequelae of lithium is increased by the concomitant use of certain conventional neuroleptics. We report two new cases of lithium neurotoxicity; one received lithium alone, not in combination with a neuroleptic. Both cases showed severe cerebellar atrophy on brain CT and MRI. Additional factors such as dehydration, systemic infection, other medications, or rapid correction of frequently-coexisting hyponatremia may contribute to the risk of lithium neurotoxicity. We discuss possible pathophysiologic mechanisms and preventive measures.