Large-vessel vasculitis (LVV) is an inflammatory condition affecting large arteries, often leading to complications such as vessel occlusion or aneurysm. Nonbacterial thrombotic endocarditis (NBTE), although rare, is generally linked with hypercoagulable states and malignancies, characterized by sterile vegetations on undamaged cardiac valves. We present a unique case of a 61-year-old woman with coexisting LVV and NBTE, evidenced by echocardiographic findings of vegetations on the aortic and mitral valves. Her initial presentation included syncope, fatigue, weight loss, and intermittent claudication. Investigations revealed elevated inflammatory markers, arterial wall thickening suggestive of vasculitis, and valvular masses on echocardiography. She responded well to anticoagulation and immunosuppressive therapy, achieving clinical remission with mild residual symptoms at two years. This case underscores the unusual overlap between LVV and NBTE, highlighting the importance of vigilant diagnostic evaluation in patients with vasculitis who present with valvular abnormalities. Given the rarity of LVV with NBTE, further studies are essential to guide optimal management for similar complex presentations.