Grisel's syndrome is a rare condition characterized by non-traumatic atlantoaxial subluxation, typically secondary to inflammatory processes or surgical interventions in the head and neck region. The literature suggests a rare incidence of approximately 1:100,000,000 per year without gender predilection.The syndrome is often associated with an abnormal head posture and can lead to serious neurological complications if not diagnosed and treated promptly. This paper reviews the pathophysiology, clinical presentation, diagnostic approach, and management strategies for Grisel's syndrome, emphasizing the importance of early recognition to prevent morbidity. A 7-year-old boy presented with torticollis, head tilt to the left, nasal obstruction, purulent nasal discharge, headache, and fever for 3 days. Examination showed painful head tilt to left, with neck rotation to the right and no neurological deficits. Nasopharyngoscopy revealed inflamed adenoid tissue with purulent discharge. CT scan showed a Fielding type I rotatory atlantoaxial subluxation. He was treated with intravenous antibiotics, analgesics, and a cervical collar. He became symptomatically better after 7 days of treatment and on follow-up, he had fully recovered and returned to normal activities. We performed a systematic review of the literature following the preferred reporting items for Systematic reviews. Literature searches were conducted in web-based search engines using MeSH terms and key words. Grisel's syndrome, though rare, should be considered in pediatric patients presenting with painful torticollis following upper respiratory infections or head and neck procedures. Prompt diagnosis through radiographic imaging and early intervention can prevent severe complications and improve patient outcomes.