Giant cell angioblastoma is an extremely rare tumor of vascular origin, described at the end of the 20th century. It belongs to tumors with intermediate malignant potential and is characterized by locally infiltrative growth. The tumor doesn’t have any clear distinctive clinical characteristics. The diagnosis is established on the basis of histological examination. Two main treatment options for this pathology are discussed in the literature: radical removal of the tumor and therapy with low doses of interferon alpha. As a rule, this is a combination treatment. This article describes our own clinical case. The patient’s parents gave their consent to the use of their child’s data, including photographs, for research purposes and in publications. Interest is in the rarity of the disease and the features of the clinical characteristics of this case, specifically the extremely unfavorable localization in the oropharynx region and, accordingly, the impossibility of carrying out not only a radical removal of the tumor, but also its resection. The high probability of developing irreversible neurological complications in this age group associated with interferon alpha therapy questioned the possibility of its use. For the first time in this histological variant of a vascular tumor, chemotherapy was applied, including metronomic therapy with cyclophosphamide and vinblastine in combination with a liposomal form of doxorubicin. After 8 courses of chemotherapy, a complete clinical response was obtained with the restoration of the patency of the respiratory and digestive tracts. The observation period at the time writing of this article was 36 months.