An 11-year-old male, who complained of hoarseness and fatigue on effort presented to our institution for evaluation. Left vocal cord paralysis and severe pulmonary hypertension was diagnosed. The patient had an enlarged pulmonary artery due to pulmonary hypertension which was responsible for compression to left vocal cord paralysis causing hoarseness. Ortner`s syndrome or cardiovocal syndrome is known as hoarseness due to left vocal cord paralysis secondary to cardiac pathologies. Although hoarseness of voice is frequently encountered in the otorhinolaryngology clinics, pulmonary hypertension related hoarseness is an unusual presentation in childhood.

Keywords: Ortner`s syndrome, childhood, heritable pulmonary arterial hypertension