High-dose arabinoside cytarabine is used as an effective regimen for post-remission acute leukemia and refractory leukemia treatment. Most commonly used regimen is 3 g/m 2 IV over 1-3 h q12 h for a total of 8-12 doses. Major toxic effects associated with cytarabine are related to bone marrow, gastrointestinal tract, and nervous system. Acute cerebellar toxicity with ataxia and dysarthria is well-known adverse effect during high-dose cytarabine therapy. This case report describes about a patient with acute myeloid leukemia on high-dose cytarabine who developed ataxia and left-sided nystagmus as cerebellar toxicity. These symptoms persisted till 2 days after the termination of high-dose cytarabine the gradually improved with steroids. Patient's good response to methyl prednisolone illustrates immune-mediated mechanism of neurotoxicity.