Ependymoma usually arises within the ventricles and central canal of the spinal cord. These tumors, found in the sellar region, are extremely rare. We report a case of pituitary ependymoma followed up over 10 years. A 59-year-old male patient presented with fatigue, general weakness, erectile dysfunction, and loss of body hair, including pubic hair. Brain magnetic resonance imaging (MRI) revealed a 3.3×3.5×2.3-cm sellar and suprasellar snowman-shaped enhancing mass. The tumor was partially resected via the trans-sphenoidal approach followed by postoperative radiation therapy. The pathologic confirmation was pituitary ependymoma. At the 10-year follow up, MRI revealed no evidence of tumor progression. With lack of knowledge about pituitary ependymoma, our case is the only case in which the disease has been well controlled over a long period of time without tumor progression.