Phenylketonurics display lower arachidonic acid levels in plasma lipids, reflecting the lower intake due to the dietary treatment poor in phenylalanine-rich animal foods. Plasma arachidonic acid levels and serum thromboxane B2 concentrations have been measured in 13 treated phenylketonuric children and compared with those of 12 healthy controls. A direct relationship between plasma arachidonic acid and thromboxane B2 concentrations has been observed only in phenylketonurics, whose plasma arachidonic acid status correlated negatively with their dietary compliance. These results suggest that the low consumption of animal fats may contribute to modulate the production of arachidonic acid-derived platelet eicosanoids.