Multiple myeloma (MM) is a malignant monoclonal proliferation of plasma cells that commonly causes pathologic fractures in the vertebrae, femur, humerus and rib. Although the association of MM and ankylosing spondylitis (AS) has rarely been reported, most of MM patients with AS share the characteristic of IgA type paraproteinemia, which suggests the presence of mechanisms that possibly connect the two conditions. In this report, we describe a 47-year-old man with a 25-year history of AS, and he was diagnosed as having IgA kappa type MM with a pathologic fracture at the left clavicle. To the best of our knowledge, this is the first case of IgA myeloma presenting with a pathologic fracture in a patient with AS. We report here on this case along conducting a review of the relevant medical literature.