Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an extremely rare variant of adult non-Hodgkin lymphomas accounting for less than 2 % of all Т-cell tumors. This disease is characterized by a very aggressive course. The overall survival of patients within 12 months after the diagnosis does not exceed 36 %. Currently, there are no standards for MEITL therapy. All plausible treatment approaches are described in very few case reports. The usual practice is to apply induction therapy regimens which are effective against the most of aggressive T-cell tumors. If stable response is achieved, patients receive high-dose chemotherapy with autologous hematopoietic stem cell transplantation. MEITL patients rather often develop various life-threatening and surgery-requiring complications. The present paper describes a case of a 50-year-old patient with MEITL. The disease was diagnosed on the basis of the immunomorphological analysis of gastric and colon mucosa biopsies. The first-line therapy consisted of 4 BV-CHEP/BV-CHP (cyclophosphamide, etoposide, doxorubicin, prednisolone, and brentuximab vedotin) cycles. The tumor progression with the development of decompensated pyloric stenosis was revealed. Laparoscopic gastroenteroanastomosis was provided. The second-line therapy included ruxolitinib and lenalidomide. The optimal combination of medication and surgery achieved the success in persistent stabilization of tumor with a satisfactory quality of life being maintained. The rarity of the disease, poor prognosis, common surgery complications, and lack of unified therapy standards raise the practical value and urgency of every single case report. MEITL patient management calls for an effective interdisciplinary interaction with personalized approach to treatment decision making in each specific clinical situation.