**Hyperammonia** is a clinically significant concept. In children of early life, any persistence of ammonia in the blood plasma is always regarded as a serious pathology. In adults, hyperammonemia is most often associated with liver failure of any etiology. In 80-90% of patients it is caused by chronic hepatitis, liver cirrhosis and congestive encephalopathy. Less often, acute infectious-toxic and toxic-allergic damage to the liver tissue can be detected in them. It is fundamentally important to differentiate the diagnosis of hyperammonemia from a hereditary urea cycle defect. Despite the general similarity of symptoms with it, in contrast to the disease caused by the production of the enzyme carbamoyltrans