A 12-year-old boy presented with right visual disturbance. Skull radiography and computed tomography (CT) showed an irregular deformity of the sella turcica, hypertrophic change of the dorsum sellae, and an inhomogeneously calcified mass in the sella turcica. Magnetic resonance (MR) imaging demonstrated the mass lesion filled the hypophyseal fossa, and extended to the dorsum sellae, right cavernous sinus, and right suprasellar region. The Dolenc pterional combined epidural and subdural approach was carried out. The histological diagnosis was chondroma. Sellar chondroma requires relief of the compression to the chiasm or optic nerve as soon as possible, so partial resection can still be beneficial. However, follow-up MR imaging or CT, visual examination, and control of pituitary dysfunction are required after the operation.