In anticardiolipin syndrome (ACS) a typical antibody constellation is associated with thrombotic and hematologic disorders. Furthermore, recurrent abortion, cerebral ischemia and different skin disorders occur. We report the case of a 29-year-old female suffering for the first time from painful, necrotic deep ulcers on the upper and lower legs and livedo racemosa on the arms as a rare example of a merely cutaneous manifestation of ACS with no demonstrable underlying disease. After systemic treatment with high-dose methylprednisolone, azathioprin and hydrocolloid dressings, healing of the scar tissue occurred. Simultaneously, a maintenance dose of acetylsalicylic acid (100 mg/day) was administered. So far, neither cutaneous relapse nor other signs of ACS have occurred.