BACKGROUND: Pituitary ependymoma is exceptionally rare. Its etiology, clinical presentation, radiologic feature, and treatment strategy are still a matter of debate. Only 7 human cases with limited data were reported in the English literature, and now we described another case of pituitary ependymoma. We also systematically reviewed previously reported cases and described its potential etiology, clinical presentation, radiologic features, pathology, immunohistochemical analysis, and ultrastructural examinations. CASE DESCRIPTION: A lesion in pituitary fossa was discovered in a 40-year-old man after suffering a progressive deterioration of vision in his right eye for >1 year with intermittent headache. The lesion was microsurgically resected and proved to be ependymoma upon pathologic and histologic examination. The patient made a fully recovery after surgery. CONCLUSIONS: To our knowledge, only 7 patients with ependymoma in the sellar region have been described in the English literature. We reported 1 more case of pituitary ependymoma and discussed the potential etiology, clinical presentation, radiologic features, pathology, immunohistochemical analysis, ultrastructural examinations, treatment, surgery, radiotherapy, chemotherapy, and prognosis of pituitary ependymoma. The case report may serve as a helpful reference for clinicians and radiologists.