Objective To observe the clinical effect of orthodontics combined with oral restoration in the treatment of congenital missing teeth. Methods A total of 56 patients with congenital missing teeth admitted to our hospital from January 2021 to December 2022 were randomly divided into control group and treatment group, with 28 patients in each group. The control group was treated with dental fixed bridge repair, and the treatment group was treated with orthodontics combined with oral restoration. The oral repair effect, dental aesthetic index and oral function were compared between the two groups. Results The total effective rate of oral repair in the treatment group was 89.29%, which was higher than 64.29% in the control group, and the difference was statistically significant (P <0.05). The tooth arrangement radian, the symmetry (width and height) of left and right upper central incisors, and the complete symmetry of left and right upper central incisors in the treatment group were better than those in the control group, and the differences were statistically significant (P <0.05). The scores of retention stability, masticatory function and occlusal function in the treatment group were higher than those in the control group, and the differences were statistically significant (P <0.05). Conclusion Orthodontic combined with oral restoration in the treatment of congenital missing teeth can achieve satisfactory oral repair treatment effect, which has a positive effect on improving the patient's tooth aesthetics and oral function, and is worthy of clinical application.