The atlantoaxial region has been extensively described as a spinal segment especially prone to injury in children. In this clinical review, the authors evaluate and summarize the management of 23 pediatric cases of atlantoaxial instability treated between March 1990 and October 2002. Four broad categories of atlantoaxial problems were observed—atlantoaxial rotatory subluxation in six patients, anterior–posterior atlantoaxial instability caused by ligamentous injury or congenital ligamentous laxity (10 patients), atlantoaxial fracture with or without dislocation (five patients), and atlantooccipital dislocation (two patients). Most cases (60.9%) were treated without surgical intervention, resulting in excellent outcomes; however, 21.7% of cases were treated with a cervical halo (mean patient age 72.6 months) alone for 3 months. Various techniques of surgical stabilization including transarticular screws with sublaminar wiring, trans-oral decompression with posterior plating, and laminectomy with Steinmann pin occipital–cervical fusion were used with good results. Both patients with atlantooccipital dislocation underwent immediate Locksley occipital–cervical fusion, with marked neurological improvement. Individualized case management must be based on clinical presentation, with internal fixation being the last resort.