Varicella-associated purpura fulminans (PF) is a rare complication to varicella infection. The condition is due to autoantibodies directed against protein S which forms part of the anticoagulation system. Lack of protein S leads to disseminated intravascular coagulation in the small vessels, which causes thrombosis and ischemia. Despite early treatment, amputation and skin-grafting is often necessary. In this case story, we give a brief review of the pathogenesis and possible modes of treatment. Knowledge of PF is necessary since early treatment may be life-saving.