There are cases where valproate has been associated with hyperammonemic encephalopathy without features of hepatic encephalopathy. The presence of significant hyperammonemia with cerebellar signs and reversal of hyperammonemia, encephalopathy and disappearance of cerebellar signs and symptoms after discontinuation of sodium valproate confirmed the diagnosis of valproate-induced hyperammonemic cerebellar ataxia with metabolic encephalopathy.