Chondro-osseous respiratory epithelial adenomatoid hamartoma is a rare hamartomatous lesion of the nasal cavity, with only five cases reported in the literature to date. We report the case of a 3-year-old boy who presented with nasal obstruction and a mass in the left nasal cavity. The mass was completely resected on endoscopy. On microscopy, hamartomatous proliferation of respiratory-type glands admixed with islands of immature hyaline cartilage, characteristic of chondro-osseous respiratory epithelial adenomatoid hamartoma, was seen. Neither local recurrence nor distant metastasis was observed after 6month follow up. Recognition of chondro-osseous respiratory epithelial adenomatoid hamartoma as a benign lesion is important to avoid unnecessary surgical procedures.