Background: Dyggve Melchoir Clausen syndrome is a rare autosomal recessive disease characterized by progressive spondyloepimetaphyseal dysplasia and mild to severe mental retardation. Atlantoaxial instability is rare in patients with the Dyggve Melchoir Clausen syndrome. Case Report: We report an uncommon association of two siblings with Dyggve Melchoir- Clausen syndrome, odontoid hypoplasia and atlantoaxial instability. Both the patients were treated with Goel’s atlantoaxial fixation procedure. 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-Melchoir-Clausen syndrome can give gratifying results.