Atlanto-axial rotatory fixation (AARF) is a rare cause of childhood torticollis 
that may occur spontaneously or in association with trauma and upper respiratory 
infections. We describe the clinical findings, as well as the effectiveness of 
imaging in the diagnosis and the treatment of 4 children with AARF, in whom 
acute fixed non-dystonic torticollis was the presenting symptom. Onset of 
torticollis was spontaneous in Case 1, after general anesthesia for 
cholesteatoma surgery in Case 2, after a trauma in Case 3, and during 
hypersomnia in Case 4. Duration of torticollis prior to diagnosis was 3 months 
in the first two patients and 20 days in the other two. All the patients 
underwent cervical X-rays examinations, which were not contributory to the 
diagnosis, followed by CT, which demonstrated C1-C2 rotatory fixation. One 
patient had a spontaneous resolution; treatment with Gardner's tongs and soft 
collar permitted restoration of the normal alignment in the other 3 patients. 
AARF must be considered in all the patients with persistent painful torticollis.