✓ The authors describe a case of arachnoiditis ossificans (AO) of the cauda equina. The lesion is a rare pathological entity usually confined to the thoracic and high lumbar regions that can cause progressive spinal cord and cauda equina compression, inducing severe neurological deterioration. The authors analyze the clinical symptoms, radiological features, histological data, and treatment options relating to this case and 13 others described in the literature; additionally, they consider the possible mechanisms responsible for ossification of the leptomeninges. Although clustered arachnoidal cells are usually implicated in its pathogenesis, an environment induced by arachnoiditis and disturbed cerebrospinal fluid flow appears to be a more important factor. A therapeutic strategy is proposed for AO for which no effective treatment currently exists.