Objective: Surgery is indicated for basilar invagination (BI) in symptomatic patients. In many patients, symptoms and signs occur due to an upward-migrated and malaligned odontoid with fixed or mobile atlantoaxial instability. Posterior distraction and fixation of the atlantoaxial joints has evolved to become the standard of care, but has some inherent morbidity. In this study, we propose that the unilateral anterior submandibular retropharyngeal approach with customized wedge-shaped titanium cages inserted into both atlantoaxial joints and anterior atlantoaxial fixation with a plate screw construct is a safer and easier option in many cases of BI. Methods: From February 2014 to February 2019, 52 patients (age range, 15-78 years; 40 males and 12 females) with symptomatic BI with atlantoaxial dislocation and minimal sagittal facetal inclination and only mild Chiari malformation without syringomyelia were offered anterior submandibular retropharyngeal atlantoaxial distraction and fixation surgery. Results: Neurological improvement occurred in 80% of patients, while the neurological status of 20% remained unchanged. No patients worsened, and no major complications or mortality was observed. Conclusion: In properly selected cases of symptomatic BI, anterior wedge cage distraction with anterior atlantoaxial fixation is a safe and simple option.