Nasal chondromesenchymal hamartoma is an extremely rare neoplasm of the nasal and paranasal sinuses. We present the case of a 10-month- old boy with a huge nasal chondromesenchymal hamartoma that was complicated by airway obstruction especially during sleep. The mass was completely resected by image-guided endoscopic surgery and confirmed histopathologically as a chondromesenchymal hamartoma.In this report, we discuss the characteristics and treatment of this unusual tumor.