Sarcoidosis is a multisystemic pathological condition of unknown etiology characterized by the formation of non-caseificating epithelioid cell granulomas that most frequently affect lymph nodes, lungs, and liver whereas spleen, skin, bones, eyes and other organs are affected less frequently. Sarcoidosis of ENT organs accounts for only 1—4% of the total number of this disease. ENT sarcoidosis with the initial intranasal manifestation is an even rarer occurrence. Patient B. aged 34 years, applied for the first time for the medical assistance to the Outpatient Department of Research and Clinical Centre of Otorhinolaryngology, Federal Medico-Biological Agency of the Russian Federation, with complaints of the difficulty of right-hand side nasal respiration and rhinorrhea. These symptoms had first appeared about 2 years ago and increased thereafter. Rhinoscopic visualization of the nasal cavity demonstrated the presence on its right-hand side of a neoplasm measuring 1.5×1.5 cm and localized near the middle nasal passage. The mucous membrane over the neoplasm remained unaltered, had dense consistency, was painless, did not shrink in response to anemisation, and did not bleed upon probing. Multislice computed tomography (MSCT) of the nasal cavity and paranasal sinuses revealed the presence on the right-hand side of the nasal cavity of a neoplasm measuring 16.5×15.5 mm and localized near the middle nasal passage that appeared to originate from the middle turbinated bone and completely obturated the lumen of the middle nasal passage. The intranasal bony structures remained unaffected. The biopsy sampling of the neoplasm was performed endoscopically under local anesthesia. The histological study confirmed that the above characteristics of the neoplasm corresponded to those of sarcoid granuloma. Conclusion. The clinical case reported in this article illustrates the possibility of the extrapulmonary manifestation of sarcoidosis starting as an intranasal pathology.