The ventriculus terminalis is a small cavity inside the conus medullaris that is formed during the embryonic development. Previous reports regarding cystic lesion of the ventriculus terminalis (CLVT) in adults have detailed a broad and diversified distribution in terms of clinical symptoms, clinical evolution, neurological findings, and treatment. Therefore, nonstandardized management has led to unsatisfactory outcomes. Thus, the authors propose a new classification system in which the clinical presentation is taken into account to standardize the cases and facilitate the proper management of these lesions. Two more cases are described. The literature was reviewed, dividing the patients into 3 groups by clinical presentation as follows: CLVT Type I, patients with nonspecific neurological symptoms or nonspecific complaints; CLVT Type II, presence of focal neurological deficit; and CLVT Type III, presence of sphincter disturbances (bowel or bladder dysfunction). Two patients were classified as CLVT Type I, 3 as CLVT Type II, and 12 as CLVT Type III. In Type I, no improvement was observed in clinical evaluation after surgery, and stable symptoms were achieved with clinical management. In Type II, 2 patients had total improvement and 1 had subtotal improvement after surgery. Finally, in Type III, 92% of the patients improved postoperatively; among these 33% presented with total improvement. Only 1 case in this group was handled conservatively and no improvement was documented. This new classification is useful to group the patients into 3 clinical types to provide guidance as to the best management options. Treatment for the Type I lesion seems to be best conducted conservatively, whereas Types II and III seem to be best handled surgically.