Essential fructosuria is a rare autosomal recessive disorder of fructose metabolism caused by a deficiency of fructokinaseenzyme activity. It is characterized by elevated fructosemia and presence of fructosuria following ingestion of fructose and related sugars (sucrose, sorbitol). Essential fructosuria is clinically asymptomatic and harmless. Dietary restriction is not indicated. The disease is MONDO_0009252 (essential fructosuria). Also known as: fructokinase deficiency, fructosuria, essential, ketohexokinase deficiency.