A case of ossification of transverse ligament of atlas (TLA) is reported. A 76-year-old female suffered from a transverse type myelopathy was successfully treated by posterior decompression. Dynamic lateral plain radiographs showed irreducible atlantoaxial subluxation (AAS). A computed tomogram revealed ossified mass compatible to ossification of TLA. Coalition of the atlantooccipital joints and osteoarthritis of the atlantoaxial joints with degenerated dens was also revealed. Magnetic resonance imaging showed compressed spinal cord at C1 level by the ossification of TLA and AAS. We suggest a mechanism of ossification of TLA as follows: hypertrophied dens and stress to the atlantoaxial joints caused by coalition of atlantooccipital joints could make forward shift of atlas leading to irreducible AAS, and continuous tension given to TLA from irreducible AAS would result in hypertrophied and ossification of TLA.