OBJECTIVE: As it was an unusual episode, our objective is to describe a case of respiratory epithelial adenomatoid hamartoma accompanied by a literature review. Clinical Case: A man presented with complaints of snoring, oral breath, and mild symptoms of allergic rhinitis. He denied other symptoms and acknowledged longtime sinusitis. During the physical exam, hypertrophy of the inferior turbinates, pallor, and a polyp on the right osteomeatal complex were noted in his nasal cavity. A CT of the paranasal sinuses was acquired, which showed maxillary and ethmoid sinus opacification on the right side, a deviated septum in the region of the right osteomeatal complex, and a bullous middle turbinate on the left. Septoplasty was indicated, and an inferior turbinate cauterization and right polypectomy were performed successfully. CONCLUSION: Hamartomas are benign tumors, non-neoplastic, characterized for glandular proliferation, and lined by ciliated epithelium with an increase in the airway epithelium, as a result of innate changes of tissue development. Its localization inside the nasal cavity is unusual. Its association with nasal polyps supports the hypothesis that inflammation may work as a trigger mechanism. The preferred treatment for respiratory epithelial adenomatoid hamartoma is surgical resection.