The primary aim of our study was to provide a comprehensive review of the clinical, imaging, and histopathological features of Tarlov cysts (TCs) and to report operative and nonoperative management strategies in patients with sacral TCs. A literature review was performed to identify articles that reported surgical and nonsurgical management of TCs over the last 10 years. Tarlov cysts are often incidental lesions found in the spine and do not require surgical intervention in the great majority of cases. When TCs are symptomatic, the typical clinical presentation includes back pain, coccyx pain, low radicular pain, bowel/bladder dysfunction, leg weakness, and sexual dysfunction. Tarlov cysts may be revealed by MR and CT imaging of the lumbosacral spine and must be meticulously differentiated from other overlapping spinal pathological entities. They are typically benign, asymptomatic lesions that can simply be monitored. To date, no consensus exists about the best surgical strategy to use when indicated. The authors report and discuss various surgical strategies including posterior decompression, cyst wall resection, CT-guided needle aspiration with intralesional fibrin injection, and shunting. In operative patients, the rates of short-term and long-term improvement in clinical symptoms are not clear. Although neurological deficit frequently improves after surgical treatment of TC, pain is less likely to do so.