Atlantoaxial dislocation with spinal canal stenosis not associated with craniovertebral junction malformations and traumatic injury is a rare pathology in children and adolescents. A clinical case of the diagnosis and surgical treatment of atlantoaxial dislocation of non-traumatic genesis combined with spinal canal stenosis in an adolescent with juvenile idiopathic arthritis is presented. A minimal instrumental fixation of the upper cervical spine allowed eliminating stenosis of the spinal canal, repairing instability of the upper cervical vertebrae, and jugulating neurological disorders.