The tumors containing ganglion cells are rare in the pituitary gland. These tumors are divided into two groups. The first group is the cases of mixed gangliocytoma-adenoma that contained both gangliocytoma and pituitary adenoma elements in the same tumor and the second group is those of gangliocytoma that contained only gangliocytoma element. Since the first description by Kiyono in 1926, 45 cases have been reported. The most common presentation of these tumors is acromegaly and the cases of Cushings disease and galactorrhea-amenorrhea also have been reported. In immunohistochemical study, hypothalamic-releasing hormones are stained in gangliocytoma cells and adenohypophyseal hormones are stained in adenoma cells. The releasing hormones stained in gangliocytoma element and the pituitary hormones stained in adenoma elements are usually closely related but unrelated cases also have been reported. We report a case of a 41 year-old lady with acromegaly which was diagnosed as mixed gangliocytoma-adenoma after surgical removal of the pituitary tumor.