We herein report a case of a 78-year-old woman who was admitted to our hospital due to a stroke with left-sided hemiparesis. Ultrasound of the carotid arteries showed a carotid body tumor on the bifurcation of the right common carotid artery, which was subsequently confirmed by a further neuroradiological investigation. Magnetic resonance imaging of the head confirmed an acute ischemic lesion located in the right periventricular region. The carotid body tumor (CBT) was surgically removed and confirmed on histopathology. Our case reveals the role of carotid ultrasound in the diagnosis of a CBT, which may be a potential cause of stroke.