To date, only 12 cases of ossifying arachnoiditis with concomitant syringomyelia have been reported in the world literature. In this article, we presented our experience of treating three patients with syringomyelia associated with ossifying arachnoiditis. Objective — based on the literature data and our own experience, we tried to define the most optimal treatment approach for this disease. Material and methods. We analyzed the data of 15 patients (12 cases reported in the literature and 3 our own cases). The diagnosis was established on the basis of complaints, medical history, patient examination, MRI and CT of the spinal cord, and intraoperative data. Progression of syringomyelia was evaluated by comparison of the neurological status and calculation of the Vaquero index before and after surgery; the Domenicucci classification was used to evaluate the severity of ossifying arachnoiditis. Surgical treatment included several stages: decompressive laminectomy, elimination of spinal cord fixation, excision of ossifying arachnoiditis, shunting of the syrinx, and repair of the dura mater. Results. An analysis of the patient data reported in the literature (12 cases) and those in our series (3) revealed that in the early postoperative period, 7 (46.7%) of these 15 patients had improvements, 5 (33.3%) patients had no changes, and 3 (20%) patients experienced a worsening. At present, there is no clear algorithm for diagnosing and treating this disease. Conclusion. Further investigation of the problem may facilitate an earlier diagnosis of the disease and provide more effective medical care.