Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening disorder characterised by arterial or venous thrombotic events, involving three or more organs in a short period of time, in the presence of persistent antiphospholipid antibodies. Long-term anticoagulation with warfarin is the standard of care to prevent recurrent vascular events. Besides supportive care, optimal management of CAPS is unclear and consensus among experts is lacking. We describe a patient with primary antiphospholipid syndrome who experienced probable CAPS after receiving rivaroxaban, resulting in extensive cutaneous ulceration, acute coronary syndrome and dialysis-dependent renal failure. Anticoagulation, glucocorticoids and plasmapheresis were started. In the haemodialysis period, he maintained treatment with long-term vitamin K antagonist. The international normalized ratio target was optimized to 3.5-4. This strategy was associated with the healing of skin lesions, regression of cardiac lesions and recovery of renal function after three years on dialysis.