Grisel syndrome is the subluxation of atlantoaxial joint as a result of infectious or inflammatory processes of the head and neck region. The etiopathogenesis of this clinical entity is not clear yet. Early interventions (antibiotherapy, cervical traction, and immobilization) are critical to avoid catastrophic outcome. Early detection of pediatric atlantoaxial subluxation is essential. It requires a combination of clinical assessment and appropriate radiographic imaging. In this report, we present a clinical and radiologic follow-up of a Grisel syndrome; by this way, we discuss the role of radiology on the diagnosis of this rare entity and preview the relevant literature.