Yo consider the clinical picture, methods of radiation diagnosis and to study the surgical results of a dermoid cyst of the nose according to the literature. A retrospective review of medical histories with the diagnosis of "Dermoid cyst of the back of the nose and nasal cavity, epidermal cyst of the back of the nose, glioma, encephalocele" was conducted from 2017 to 2022 in the Pediatric Otorhinolaryngological Department of the National Medical Research Center for Otorhinolaryngology of the Federal Medical-Biological Agency of Russia. The case histories were analyzed by the nature of the lesion, the imaging techniques performed, the course of the operation and the results obtained. A total of 16 medical histories were analyzed, the average age was 4.5 years (range 10 months - 15 years), over the past 5 years with a diagnosis of "Dermoid cyst of the nasal dorsum and nasal cavity, glioma, epidermal cyst of the nasal dorsum, encephalocele". All patients underwent magnetic resonance imaging (MRI) in the preoperative period, 14 patients also underwent computed tomography (CT). Of these, 7 had a confirmed dermoid cyst with a fistula, 3 patients without a fistula, 3 patients had glioma, and 1 had encephalocele, 2 patients had an epidermoid cyst. A fistulous opening of the dermoid cyst of the nasal dorsum and nasal cavity was observed in the upper third of the nasal dorsum in 3 children, in the middle third in 2 patients and in the lower third in 2 children. The article presents a scheme for the characteristics of the lesion and the tactics of surgical treatment in comparison with the data of foreign authors. Intraoperatively, intracranial spread occurred in 6 patients. Various surgical approaches for intracranial proliferation and a corresponding literature review are also presented. Catamnestic follow-up ranged from 1 to 5 years (on average, 3.5 years), no relapses or postoperative complications were noted. Nasal median heterotopias are a rare congenital anomaly. Preoperative preparation should include CT and MRI to assess the lesion and exclude intracranial spread. The surgical approach depended directly on the localization of heterotopia and its spread. All patients had a good cosmetic result after the surgical treatment performed by us according to the author's method.