Rosette-forming glioneuronal tumour (RGNT) is a rare primary tumour of the central nervous system with a low degree of malignancy. Using such databases as PubMed, Web of Science and Google Scholar as well as terms “rosette and forming and glioneuronal”, we found 153 RGNT cases dated 2002-2020. Low occurrence of the disease and absence of specific symptoms make it possible to perform a differential diagnosis judging from the clinical picture. Clinical manifestations of the disease are rather variable and depend on the tumour location. Magnet-resonance tomography is the leading method for diagnosis of such disease. The article presents a case of successful treatment of a patient RGNT of the fourth ventricle. Subtotal resection of the tumour was performed due to its diffuse growth. Tumour residue is subject to dynamic follow-up observation
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