Perineurial (Tarlov) cysts are meningeal dilations of the posterior spinal nerve root sheath that most often affect sacral roots and can cause a progressive painful radiculopathy. Tarlov cysts are most commonly diagnosed by lumbosacral magnetic resonance imaging and can often be demonstrated by computerized tomography myelography to communicate with the spinal subarachnoid space. The cyst can enlarge via a net inflow of cerebrospinal fluid, eventually causing symptoms by distorting, compressing, or stretching adjacent nerve roots. It is generally agreed that asymptomatic Tarlov cysts do not require treatment. When symptomatic, the potential surgery-related benefit and the specific surgical intervention remain controversial. The authors describe the clinical presentation, treatment, and results of surgical cyst fenestration, partial cyst wall resection, and myofascial flap repair and closure in a case of a symptomatic sacral Tarlov cyst. They review the medical literature, describe various theories on the origin and pathogenesis of Tarlov cysts, and assess alternative treatment strategies.