Cardiovocal syndrome is left recurrent laryngeal nerve palsy associated with cardiovascular disease. Herein, we report a rare case of left recurrent laryngeal nerve palsy caused by idiopathic pulmonary arterial hypertension. A 40-year-old woman diagnosed with idiopathic pulmonary arterial hypertension was referred to our department for occult infection foci in the ear, nose, and throat (ENT). She had no apparent subjective symptoms in the ENT area, including hoarseness. Flexible laryngoscopy revealed left vocal cord paralysis, and contrast-enhanced computed tomography revealed dilatation of the pulmonary trunk, bilateral pulmonary arteries, and right ventricle, suggesting compression of the left recurrent laryngeal nerve. In our daily practice, when we encounter a left recurrent laryngeal nerve palsy that seems to be endogenous, cardiovascular lesions should be ruled out.