Arachnoid cysts are fluid-filled duplications or splittings of the arachnoid layer with a content which is similar but not equal to the cerebrospinal fluid. Arachnoid cysts are not actual neurodegenerative disorders, rather the underlying defect of the texture of the arachnoid layer is probably congenital in nature. They can occur sporadically or can be associated with other malformations or diseases. Arachnoid cysts may be discovered in early childhood. However, they can develop de novo, grow or decrease in size. They may be diagnosed by ultrasound screening in the fetal period or be discovered during childhood or adulthood. Many arachnoid cysts are asymptomatic.Treatment strategies are discussed controversially. If they are diagnosed incidentally or are correlated with only very mild symptoms, a conservative management with follow-up imaging may be favored. If they grow, they can cause headaches, seizures or other neurological symptoms and require neurosurgical treatment. This chapter addresses aspects of pathogenesis, clinical symptoms, indication for neurosurgical treatment and treatment options.