Spindle cell oncocytoma (SCO) is a rare tumor of adenohypophysis, arising from the sellar region. So far, about 35 cases of SCO in the sellar region have been reported. In this report, we present the first case of pediatric SCO and review the literature concerning the tumor origin, clinical presentations, radiological features, and treatment modalities. An 8-year-old male was referred to our clinic with progressive visual loss in the left eye and headache over the past 6 months. Cranial magnetic resonance imaging revealed a solid adenohypophysis mass with suprasellar extension, as well as compression and displacement of the optic chiasm. The patient underwent endoscopic trans-sphenoidal resection of the tumor. The tumor was diagnosed as SCO based on the histological study. He did not receive radiation therapy. The patient's condition remained stable, with no radiological recurrence in the past follow-up 2 years after the surgery.