Coagulation abnormalities are not included in the definition of adult onset Still's disease (AOSD). Nevertheless, eight cases with disseminated intravascular coagulopathy (DIC) sometimes associated with hepatopathy have been reported in the literature. The first patient, a 39-year-old woman, presents an AOSD well stabilized under steroid therapy since 1986. In 1989, she develops a DIC, a pancytopenia and a non-specific hepatopathy with a good response to an intensive steroid therapy and heparin. The second patient, a 30-year-old woman, presents since the age of three years, with juvenile Still's disease in remission since the age of 19. At the age of 28, she presents an AOSD, complicated by DIC which necessitated the introduction of methotrexate given for the poor response to high doses of steroids. The etiology of AOSD and DIC is still unknown. An iatrogenic cause (salicylates, indomethacin, chrysotherapy) has been suggested. An AOSD with DIC (sometime associated with an important hepatopathy and/or pancytopenia) should be treated early with corticosteroids and heparin in order to prevent a possible lethal outcome.