Introduction: Aortic mural thrombosis associated with a malignant disease is rare, and whether anticoagulation therapy or surgical treatment is the more definitive primary treatment remains uncertain. This study aims to determine the best treatment strategy for aortic thrombosis in a patient with a malignant disease. Methods: We reviewed medical literature using the PubMed database and present a case of aortic thrombosis due to a hypercoagulable state related to sigmoid colon adenocarcinoma. Results: Of the 18 patients from 14 articles included in this study, 13 received simple anticoagulation as a primary treatment (anticoagulation group), while 5 underwent surgical treatment (surgical treatment group). Recurrence or exacerbation of embolism was found in 2 (15.4%) of the 13 patients and in 1 (20.0%) of the 5 patients (P ¼ 1.0). Major complications were observed in 1 (7.7%) of the patients in the anticoagulation group and in 1 (20.0%) of the 5 patients in the surgical treatment group (P ¼ .49). No significant differences between the groups were found. Conclusions: A simple anticoagulation therapy may be as effective as surgical treatment in patients with aortic thrombosis associated with malignancy.