The present systematic review examined the effectiveness of weight management interventions comparing diets with varying macronutrient distributions on BMI and cardiometabolic risk factors in overweight or obese children and adolescents. A systematic search of seven databases for the period 1975-2013 identified 14 eligible randomized or quasi-randomized controlled trials conducted with 6-18-year-old subjects. Seven trials compared a low-fat (≤ 33% energy or < 40 g/day) to an isocaloric (n = 2) or ad libitum (n = 5) low-carbohydrate diet (< 20% energy or < 60 g/day). Meta-analysis indicated a greater reduction in BMI in the low-carbohydrate group immediately after dietary intervention; however, the quality of the studies was limited and cardiometabolic benefits were inconsistent. Six trials compared increased-protein diets (19-30% energy) to isocaloric standard-protein diets (15-20% energy) and one compared an increased-fat diet (40% energy) to an isocaloric standard-fat diet (27% energy); there were no differences in outcomes in these studies. Current evidence suggests that improved weight status can be achieved in overweight or obese children and adolescents irrespective of the macronutrient distribution of a reduced-energy diet. Tailoring the macronutrient content to target specific cardiometabolic risk factors, such as a low-carbohydrate diet to treat insulin resistance, may be possible, but further research is needed before specific recommendations can be made.