Ammonia is a neurotoxin that is normally cleared by the intact liver and if not, hyperammonemia results in hepatic encephalopathy. Hyperammonemia may be owing to primary or secondary causes. Early diagnosis is important to prevent permanent brain damage. Advanced malignancy involving the liver is associated with hyperammonemia as a result of abnormality of the portal venous system or massive hepatic tumor burdon. Neuroendocrine tumors are an example of a malignant process that frequently involves the liver but despite this, may still have a relatively good prognosis, and are often characterized by chronic manageable symptoms and slow progression. Hyperammonemia in neuroendocrine tumor would represent a potentially reversible but ongoing process associated with an indolent malignancy. We present 2 cases that are examples of this diagnosis and discuss the diagnostic and management issues that may arise.