Hamartoma is an extremely rare congenital malformation of the larynx. Hamartomas of the nasal cavity or nasopharynx are classified as epithelial, mesenchymal, and mixed epithelial and mesenchymal types. Presenting symptoms result from airway obstruction and may include slowly rising respiratory distress, stridor, changes in voice, eating, and activity levels. We present a 1-day-old newborn with a history of stridor and respiratory distress caused by a polypoid mass on the anterior half of the left ventricular band. We performed an excisional biopsy under direct laryngoscopy. Histopathologic finding after excisional biopsy was consistent with hamartoma. We report and discuss the pathological features and differential diagnosis of this rare laryngeal hamartoma. To our knowledge, laryngeal hamartoma presenting with stridor has not been described in the literature thus far.