Chronic periaortitis (CP) is a rare disease, the common denominator of which is a retroperitoneal periaortic fibroinflammatory tissue that frequently obstructs neighboring structures. CP has already been reported in association with various autoimmune disorders, such as Hashimoto's thyroiditis, systemic lupus erythematosus and Sjögren's syndrome. The association with autoimmune disorders suggests that CP is a manifestation of a systemic autoimmune process. We describe the first case of antiphospholipid syndrome in a patient with CP.