Congenital arachnoid cysts (CAC) are benign developmental disorders. A number of theories have been proposed to explain the origin and progression to the symptomatic stage. Management strategies range from not treating the asymptomatic cysts to craniotomy, excision of cyst wall and establishment of a communication with the adjacent subarachnoid cistern or ventricle. Other lesions may resemble CAC, and can be differentiated from CAC only by a critical review of the biopsy specimen. We have reviewed our experience with true CAC and similar lesions. Based on our experience we recommend craniotomy, excision of cyst wall and establishment of a communication with an adjacent subarachnoid cistern or ventricle as the primary procedure.