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Sellar gangliocytomas are exceedingly rare, well-differentiated, low-grade neoplasms that frequently occur in the presence of a pituitary adenoma (PA). We report a case of a sellar gangliocytoma coexisting with growth hormone (GH) secreting PA. A 43-year-old man was brought to our hospital with right-eye visual disturbances for 2 months, along with headaches, acromegaly, temporal hemianopia in the right eye, and recent onset of Type 2 diabetes mellitus. Endocrinological studies found elevated serum level of GH. A computed tomographic scan showed a mass in the sellar region with suprasellar extension and thus a diagnosis of an expanding pituitary macroadenoma was established. It was removed by transnasal transsphenoidal surgery. On histopathological examination of the resected specimen, diagnosis of mixed gangliocytoma-PA was confirmed as it showed two distinct morphological components of the tumor comprising neoplastic ganglionic cells and adenomatous cells.