Nonbacterial thrombotic endocarditis, a form of noninfectious thrombotic endocarditis, is mainly characterized by deposition of sterile platelet thrombi on heart valves. Usually, it is observed in advanced malignancy. Herein, we report a case of a previously healthy male with recent unprovoked deep vein thrombosis presented with acute ischemic stroke. The echocardiogram revealed aortic and mitral valve masses. Eventually, he was discovered to have advanced cholangiocarcinoma. The present case, apart from being the youngest reported case, is among the few reported cases which manifest the association between cholangiocarcinoma and nonbacterial thrombotic endocarditis.