Objective. To present a clinical case of arachnoiditis ossificans associated with syringomyelia and a brief literature review with an emphasis on its etiology, pathogenesis and methods of diagnosis and treatment.Material and Methods. A clinical case of a 68-year-old patient with symptomatic arachnoiditis ossificans is described. The diagnosis was established on the basis of the results of intraoperative biopsy, histological examination of the resected fragment and confirmed in the postoperative period using CT of the spinal cord, multislice CT myelography, etc. Analysis of the course of the pathology raised the following questions: diagnostic criteria and optimal tactics for treating this disease. A brief review of cases of arachnoiditis ossificans described in the literature for the period from 1982 to the present is given.Results. A review of cases of ossifying arachnoiditis described in the literature showed that today there is no single tactic for diagnosing and treating this disease. In most cases, the diagnosis is established intraoperatively (65 % of analyzed cases). At the preoperative stage, CT provides reliable visualization of ossification. When choosing therapy, it is necessary to be based on the severity of the clinical picture, the degree of ossification of the arachnoid membrane and the presence of concomitant pathology of the affected spinal cord department (such as syringomyelia).Conclusion. The presence of a growing neurological deficit should be considered an indication for surgical treatment of patients with arachnoiditis ossificans. The goals of surgery should include decompression of neural structures and restoration of normal cerebrospinal fluid circulation.