We describe the case of a 70-year-old male with the catastrophic antiphospholipid syndrome admitted for skin gangrene of the fingers. The initial diagnosis was antiphospholipid antibody syndrome in a patient with rheumatoid arthritis and a history of deep vein thrombosis of the lower limbs. Liver involvement, the characteristic skin gangrene, pneumonia and worsening severe renal failure were determinant to make the final diagnosis of catastrophic antiphospholipid syndrome that led the patient to death.