Occipitoatlantoaxial rotatory fixation (OAARF) is a rare condition involving fixed rotational subluxation of the atlas in relation to both the occiput and axis. Atlantoaxial rotatory fixation (AARF) appears to precede OAARF in most cases, as untreated AARF may cause compensatory counter-rotation and occipitoaxial fixation at an apparently neutral head position. We report a case of OAARF in an 8-year-old girl with juvenile idiopathic arthritis. Cervical imaging demonstrated slight rightward rotation of the occiput at 7.63° in relation to C-2 and significant rightward rotation of C-1 at 65.90° in relation to the occiput and at 73.53° in relation to C-2. An attempt at closed reduction with halo traction was unsuccessful. Definitive treatment included open reduction, C-1 laminectomy, and occipitocervical internal fixation and fusion.