Infective endocarditis (IE) is a rare infection of the inner lining of the heart and valves, mainly affecting those with pre-existing heart problems. Patients usually present with fever and other non-specific systemic symptoms such as malaise, myalgia, and night sweats. However, IE may have unusual presentations, making its diagnosis even more challenging. Here, we report an unusual case of IE presenting as confusion.A 51-year-old man presented to the emergency department complaining of confusion for three days. Upon physical examination, there was an evident holosystolic murmur at the apex radiating to the axilla and an early decrescendo diastolic murmur at the left lower sternal border. Laboratory tests including white blood cell count and C-reactive protein were elevated. Transthoracic and transesophageal echocardiogram showed severe mitral regurgitation and aortic regurgitation, in addition to the presence of a mobile mass suspected to be vegetation on each of the mitral and aortic valves. Magnetic resonance imaging of the brain was performed which revealed ischemic lesions of possible embolic origin. Mitral and aortic valve replacement was performed successfully, and the patient recovered well.Our case emphasizes the possibility of unusual presentations in patients with IE, with confusion being one of them. It is important for physicians to always consider the diagnosis of IE in patients presenting with neurological symptoms of unclear origin.