• Ten children with classic phenylketonuria (PKU) participated in a controlled study of a phenylalanine-free formula recently released in the United States (PKU-2). Control data were obtained in the clinic while the children were receiving their baseline formula. The children were given the study formula and returned to the clinic for follow-up after they had been receiving the new formula for 4, 8, and 12 months. Serum phenylalanine and tyrosine concentrations, other hematologic measurements, urine analysis, growth, electroencephalogram, and physical findings remained similar to baseline values throughout the study. Nutrient intakes were comparable with the exception of a decrease in several micronutrients relative to baseline levels. We found that PKU-2 is appropriate for children with PKU who are over 3 years of age and may prove beneficial for the overweight child with PKU.