Grisel’s syndrome is a rare cervical spine disorder characterized by non-traumatic rotary subluxation of the atlantoaxial joint. This article provides a systematic review to collect evidence on its pathogenesis, clinical manifestations, diagnosis, treatment, complications, and risk management, in order to guide clinical diagnosis and treatment. The syndrome is often associated with adenotonsillectomy. Patients typically present with neck stiffness, pain, and a “cock robin posture” (chin tucked in and head tilted forward). Diagnosis relies on MRI and CT scans. After timely diagnosis, most patients can control the condition through conservative treatment. However, those with ineffective conservative treatment or severe subluxation may require surgical intervention. Therefore, early diagnosis and treatment are crucial. This article focuses on the progress in the diagnosis and treatment of Grisel’s syndrome after adenotonsillectomy, which will provide new insights into the diagnosis and treatment of this rare disease.