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This is a report of two patients who were diagnosed to have NF-1. The patients had severe dystrophic soft tissue and bone changes leading to craniovertebral junction and subaxial cervical spinal instability and deformity. Both the patients underwent atlantoaxial and subaxial cervical spinal stabilization. No bone, soft tissue or tumor resection was done for decompression. Both patients had gratifying clinical recovery. Follow-up in both the patients is more than 12 months.

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AIM:To discuss the rare association of atlantoaxial instability in patients with the Dyggve-Melchior-Clausen syndrome, a rare autosomal recessive disease characterized by progressive spondyloepimetaphyseal dysplasia and mild to severe mental retardation. MATERIAL and METHODS:We report an uncommon association of two siblings with Dyggve-Melchior-Clausen syndrome, odontoid hypoplasia and atlantoaxial instability. Both the patients were treated with Goel's atlantoaxial fixation procedure. RESULTS:The patients had a remarkable neurological recovery following the stabilization procedure. CONCLUSION:Atlantoaxial instability is a potentially life-threatening condition in patients with this syndrome and should be treated early with atlantoaxial stabilization. Recognition and treatment of atlantoaxial instability in patients with Dyggve-Melchior-Clausen syndrome can give gratifying results.