Benign and malignant formations of the nose and paranasal sinuses in the early stages of development are asymptomatic or manifest minor nonspecific signs, and therefore often remain unrecognized. Hamartoma is a tumor-like formation of a dysembriological nature, consisting of excessive tissues peculiar to the affected organ. Due to the rarity of the occurrence of hamartomas of the sinonasal region, especially in pediatric practice, their diagnosis may present certain difficulties, since they can mimic other formations, such as nasal polyps or inverted papilloma, may occur as an isolated formation in the nasal cavity, or in combination with chronic rhinosinusitis, nasal polyposis, allergic rhinosinusitis. Aim – to report a rare clinical case in pediatric practice of respiratory epithelial adenomatoid hamartoma associated with allergic rhinosinusitis and polyposis, display the relationship between the occurrence of respiratory epithelial adenomatoid hamartoma and chronic inflammation of the nasal cavity and paranasal sinuses, to carry out differential diagnosis with other exophytic aggressive lesions of the nasal cavity, combine and summarize available data on sinonasal hamartomas. The article describes a clinical case of respiratory epithelial adenomatoid hamartoma in a 17-year-old child with a previous 4-year follow-up with nasal cavity polyposis and a history of repeated polypotomy. The results of endoscopy and computed tomography of the nasal cavity and paranasal sinuses, the results of surgical intervention, as well as the conclusions of a histological examination of the tumor removed from the patient are presented. The authors analyzed the domestic and foreign literature, on the basis of which they presented a differential series of sinonasal hamartomas. Respiratory epithelial adenomatoid hamartoma is a rare pathological formation of the sinonasal region, which manifests itself as an isolated polypoid mass in the nasal cavity or as an accidental surgical finding in patients with chronic sinusitis. The association with nasal polyps supports the hypothesis that inflammation may be one of the inducing factors. Respiratory epithelial adenomatoid hamartoma, although rare, should be considered in the differential diagnosis of exophytic lesions of the nasal cavity. The tactics of treatment involves endoscopic removal of the formation within healthy tissues, which provides good long-term results, relapses are extremely rare.