Atlanto-occipital rotatory dislocation (AORD) has rarely been reported in the literature; for this reason, the clinicoradiologic characteristics of this injury are not well described. We describe the case of a 67-year-old man who sustained a cervical spine trauma. He reported only neck pain and was neurologically intact. A computed tomography scan showed a rotatory displacement of the atlanto-occipital joints associated with a widened condylar–C1 interval; in addition, magnetic resonance imaging showed injuries to the stabilizing ligaments of this area. A systematic literature review was also performed to identify previous cases of patients with AORD. The patient was treated with craniocervical fixation from occipital to C1, achieving a good outcome. The literature review yielded 9 cases of patients with AORD. Compared with patients with atlanto-occipital dislocation, patients with rotatory dislocations have a less severe degree of displacement of the atlanto-occipital joints and better clinical outcome. Compared with previously classified atlanto-occipital dislocations, AORD is an independent and unique variation. AORD presents with different biomechanical, clinicoradiologic, and prognostic characteristics and represents an important addition to the spectrum of atlanto-occipital dislocation injuries.