Introduction Many etiologies can lead to atlantoaxial subluxation. In Grisel syndrome, this subluxation occurs spontaneously after inflammatory processes of the head and neck. Nonsurgical treatment most often resolves the symptoms; however, in some cases, surgical treatment is necessary to repair the subluxation. Various surgical techniques and instrumentation systems have been used to treat atlantoaxial subluxation, although there is no consensus regarding the best treatment method for the pediatric population. We aim to describe a modified surgical technique for the treatment of atlantoaxial subluxation in a child with Grisel syndrome. Patient and Methods: Our case study involves a 5-year-old female patient with a 6-month history of unresolved Fielding type II atlantoaxial subluxation because of the Grisel syndrome. Despite conservative treatment, the patient's symptoms continued to progress. After two failed closed reduction attempts, open reduction and C1–C2 fusion with a modified Goel–Harms technique was performed with atlas laminar hook and axis pedicle polyaxial screws. A literature review of the surgical treatment of the Grisel syndrome was also performed. Results After surgery, the patient exhibited full clinical and functional recoveries with complete resolution of symptoms. At the 24-month follow-up examination, there was continual evidence of satisfactory reduction and fusion. No complications were observed. On completion of the literature review, seven Grisel syndrome cases were found to have been treated surgically with the minimum patient age being 9 years. Conclusion Conservative management of Grisel syndrome is the most common and effective treatment; however, a few surgical cases have been reported in the literature with good results. We exhibit a safe, new surgical construct for pediatric patients in the treatment of atlantoaxial subluxation and instability, with satisfactory clinical results and solid fusion at 24 months postsurgery.