The pathogenesis of endodermal cysts of the posterior fossa is still incompletely understood. The authors reviewed three new cases and those reported in the literature to clarify the clinical, pathological, radiological, and surgical characteristics of these lesions. A total of 49 cases were reviewed. Details on demographic profiles, clinical characteristics, histopathological and radiological features, and surgical methods were collected. These cysts have a predominance in male patients (61%) and can occur at any age (birth-77 years). In patients with posterior fossa endodermal cysts there is a bimodal age distribution and headache is the most frequent complaint. On immunohistopathological examination, endodermal cysts were reactive for epithelial membrane antigen and for keratin immunostains whenever the latter were tested. The cysts were reactive for carcinoembryonic antigen in nine of 11 cases. Endodermal cysts were located anterior to the brainstem in 51% of cases and in the fourth ventricle in 21% of cases. They frequently appeared hypodense on computerized tomography scans, and in five cases, the lesion was missed. The cyst's appearance on magnetic resonance imaging is variable. Resection was complete in 19 cases and partial in 11; marsupialization was achieved in two cases. Three recurrences have been reported. Total excision with preservation of neurological function should be the goal. Cranial base approaches are helpful for surgical access in selected examples of these lesions.