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Angiosarcoma of the heart is an uncommon soft tissue sarcoma. A few cases of disseminated intravascular coagulation (DIC) associated with angiosarcoma occurring in various organs, but not the heart, have been reported. Although taxane is commonly used in the treatment of metastatic angiosarcoma, data on the efficacy of nab-paclitaxel for angiosarcoma are limited. Here, we report probably the first case of a patient with primary cardiac angiosarcoma with coexisting DIC who was successfully treated with nab-paclitaxel. A 62-year-old female with chief complaints of nausea and shortness of breath was diagnosed as having cardiac angiosarcoma with liver metastases. Four months after the resection of her primary tumor, the hepatic metastatic lesions progressed rapidly accompanied by new metastatic lesions in the right iliac bone and signs of DIC. She received nab-paclitaxel as first-line chemotherapy. A response of stable disease was achieved after 2 treatment cycles and DIC was successfully controlled for at least 4 months. This report suggests potential utility of nab-paclitaxel for angiosarcoma complicated with DIC. We also review the literature for all cases of angiosarcoma with DIC reported so far.