Abstract We report a case of a 42-year-old man with antiphospholipid syndrome (APS) with chondritis. He presented with preceding insidious progressive occlusion of the bilateral common iliac arteries extending to the lower two-thirds of the abdominal aorta. Active thrombotic events developed concurrent with the onset of chondritis, and resulted in massive thromboses in multiple organs and renal dysfunction. Both conditions responded well to combined intravenous high-dose methylprednisolone and anticoagulation therapy. The inflammatory component of his disease may have played a major role in the pathogenesis of thrombosis given the concurrent active inflammation from his chondritis.