To evaluate whether a coupled family history of diabetes (FHD) and low birth weight (LBW) or high birth weight (HBW) is associated with metabolic syndrome (MetS) in children and adolescents.A total of 1262 children and adolescents age 7-15 years were randomly selected to enroll in this cross-sectional, community-based study.In the overall population, HBW (odds ratio [OR] = 1.4; 95% confidence interval [CI] = 1.2-10.9), but not LBW (OR = 0.97; 95% CI = 0.6-2.1), was significantly associated with MetS. In the group without FHD, HBW (OR = 1.730; 95% CI = 1.1-2.7), but not LBW (OR = 1.139; 95% CI = 0.7-23), was associated with MetS. In the group with FHD, both LBW (OR = 2.690; 95% CI = 1.4-15.1) and HBW (OR = 3.289; 95% CI = 1.3-30.6) were associated with MetS. Both LBW (OR = 4.710; 95% CI = 1.4-39.7) and HBW (OR = 3.127; 95% CI = 1.3-45.1) were associated with MetS in children and adolescents with FHD in the maternal branch but not in the paternal branch.HBW or LBW, in combination with positive FHD in the maternal branch, are determinants of MetS.