This cohort study was conducted to examine the association of low carbohydrate diet (LCD) score with risk of incident metabolic syndrome (MetS) and its components after 3.6 years of follow-up in 2044 healthy adults within framework of the Tehran Lipid and Glucose Study. The LCD was calculated according to intakes of carbohydrate, monounsaturated fatty acids, refined grains, vegetable protein, fiber, n3/n6 polyunsaturated fatty acids ratio and glycemic load (GL), using a food frequency questionnaire at baseline. In the final model, after adjustment for age, gender, smoking status, physical activity and energy intake, there was significant association between LCD score and fasting plasma glucose (beta: -0.002, 95%CI: -0.005, -0.001), triglycerides (TGs) (beta: -0.002, 95%CI: -0.004, -0.001), BP (beta: -0.001, 95%CI: -0.002, 0.000), WC-BMI (beta: -0.003, 95%CI: -0.005, -0.001) and MetS risk Z-scores (beta: -0.015, 95%CI: -0.021, -0.002). In conclusion, LCD may be associated with a decreased risk of MetS and its components in adults.