Background Disseminated Intravascular Coagulation (DIC) after Endo-Vascular Aneurysm Repair (EVAR) is a highly uncommon clinical entity. With only a few case-reports available, its management strategy still remains enigmatic. Objectives The purpose of this study was to retrieve, synthesize, and appraise all existing data for DIC after EVAR. Materials and methods All published articles regarding DIC post-EVAR were identified from 3 major databases and analyzed. Clinical parameters, predisposing factors, along with mortality and morbidly were assessed. Results The total number of publications included in the review was 15 describing 17 cases. DIC presented with a broad spectrum of clinical manifestations, while the time of diagnosis varied significantly. Endoleak was the main causative factor, with an incidence reaching 71%. The mortality of DIC after EVAR reached 29%, regardless of the therapeutic approach chosen. DIC was treated effectively in 47% of the patients (8/17), with better outcomes among patients who received conservative therapy or among those who were submitted to endovascular interventions. Conclusions DIC after EVAR, although rare, is a potentially lethal clinical condition which requires prompt diagnosis and urgent medical consideration. Treatment of endoleak may help in quick restoration of normal parameters