This review article highlights current diet therapies available for treatment of a rare genetic disorder, phenylketonuria which has grown to be prevalent in all parts of the world based on numerous epidemiological surveys. This disease is characterized by defective phenylalanine ammonia lyase leading to toxic accumulation of phenylalanine in the blood as well as blood brain barrier resulting in epileptic seizures, low intelligence quotient, mood swings, pigmentation, mental retardation and other behavioural and psychological issues. The review article discusses not only about the current prospects of treatment that is available in the market including medicated diet formulations, glycomacropeptides and large neutral amino acids but also sheds light on the complications associated with each of the therapies. Furthermore, financial perspective is drawn towards each of the available treatment from the viewpoint of the patients suffering from phenylketonuria. Therefore, the need of alternative, cost-effective and sustainable solutions is emphasized for tackling the issue in order to meet the growing demands in the given future and also stress on the need to develop novel R&D solutions and strategies to develop therapeutic diet therapy based products for phenylketonuria across the world.