Infective endocarditis (IE) is a critical infection with a high mortality rate, and it usually causes sepsis. Though disseminated intravascular coagulation (DIC) sometimes occurs in IE patients, no definitive treatment strategy for IE patients with DIC as a complication exists. Therefore, we evaluated the prevalence, surgical results and treatment strategy for IE complicated with DIC. Between 2009 and 2017, a total of 585 patients undergoing valve surgery for active IE were enrolled at 14 institutions, of whom 116 (20%) had DIC as a complication. For further evaluation, we divided DIC patients into medical treatment-first (n = 45, group M) and valve surgery-first (n = 51, group S) groups after excluding 20 patients with intracranial haemorrhage. The overall survival rates at 1 and 5 years were 91% and 85% in the non-DIC group and 65% and 55% in the DIC group, respectively (P < 0.001). Recurrence-free survival rates at 1 and 5 years were 99% and 95% in the non-DIC group and 94% and 74% in the DIC group, respectively (P < 0.001). The overall survival rates at 1 and 5 years were 77% and 64% in group S and 51% and 46% in group M, respectively (P = 0.032). Multivariable analysis revealed that 'medical treatment first' was an exclusive independent risk factor [hazards ratio 2.26 (1.13-4.75), P = 0.024] for overall mortality. Mortality and IE recurrence were statistically significantly higher in DIC patients. Valve surgery should not be delayed because most patients proceeding with medical treatment eventually require emergency surgery and their clinical outcomes are worse than those of patients undergoing early surgery.