The mixed gangliocytoma-pituitary adenoma is a very rare intracranial neoplasm, and it is frequently associated with endocrine symptoms; acromegaly, Cushing's disease, galactorrhea and amenorrhea. Morphologically it shows a mixture of gangliocytoma and pituitary adenoma in various proportions. In the area of gangliocytoma, there is no neoplastic glial component. The portion of pituitary adenoma consists mainly of chromophobe cells. There are three hypotheses in its histogenesis. We report a case of a 41 year-old lady presented with acromegaly and amenorrhea existing for 3 years. On magnetic resonance image, there is a dumbell-shaped mass in the sellar region. Histologically it showed typical features of mixed gangliocytoma-pituitary adenoma. This case is presented in the view of its rarity and interesting possible histogeneses.