Anterior thoracic intradural arachnoid cysts (ATIACs) are a rare cause of spinal cord and nerve root compression, for which different treatment strategies have been proposed. Although ATIAC represents a well-known clinical entity, the choice of surgical method has not been uniform, and no study has been specifically designed to compare the results of the different treatment options adopted. The authors report the case of a 40-year old man with a 1-year history of dorsal pain, weakness in the lower extremities, gait disturbance, and mild sexual and urinary dysfunction. On neurological examination spastic paraparesis, lower-extremity hypertonia, and hypesthesia below T-2 were demonstrated. Magnetic resonance imaging revealed the presence of an ATIAC at the T-2 level. The patient underwent complete microsurgical removal of the cyst. The authors conducted a Medline search of the relevant literature from 1966 to 1998 and also obtained data on other cases in which patients underwent surgical treatment of ATIAC. The literature search yielded five such cases. Treatment strategies were complete excision and fenestration followed by placement of a shunt. In addition, one case was characterized by intraoperative cyst rupture during retraction of the spinal cord. Correct preoperative workup coupled with microneurosurgical technique allow for successful removal of the lesion and excellent outcome. Based on the literature review and the results in our case, the complete excision of ATIAC is associated with an excellent outcome, which is different from results achieved using other surgical strategies.