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BACKGROUND: Acute disseminated intravascular coagulation (DIC) in cancer is rare and often fatal, whereas chronic forms are more frequent. CASE REPORT: A 37-year-old man with a known history of melanoma excised 6 years ago, presented with abdominal pain, fatigue and epistaxis. A computed tomography scan revealed diffuse metastatic disease of the liver, and laboratory analysis was consistent with accentuated DIC. After chemotherapy with dacarbacine, vinblastine and cisplatine, the acute DIC improved rapidly. After a second cycle, the tumor marker S-100 dropped from 15 to 3.1 microg/l (< 0.15 microg/l), suggesting that the improvement of the DIC was associated with a response to chemotherapy. After 2 cycles of chemotherapy, a palliative right hemihepatectomy was performed followed by 4 additional postoperative chemotherapy cycles. The patient survived 1 more year without relapse of the DIC. CONCLUSION: The cornerstone of managing DIC in cancer consists in treatment of the underlying disease. The rapid control of DIC in our patient can be explained by early diagnosis of the coagulopathy and the fast response of the tumor to the multidrug therapy. Thus, combination chemotherapy of metastatic melanoma is justified if an effective response has to be obtained in a limited time.