Gangliocytoma of the sellar region are extremely rare and often coexist with pituitary adenoma. Aim of this review is to collect all the cases reported in literature and to summarize the most recent literature evidences on the subject. A systematic review was performed through PubMed searching for articles describing gangliocytoma of the sellar region in the adult population, up to September 2015. An analysis of epidemiological data, clinical presentation, histochemical and radiological features and follow-up data was performed. A total of 55 articles were included in our analysis, reporting 129 cases of gangliocytoma of the sellar region. One case identified in our institution was also included. 85% of cases presented in association with pituitary adenomas. A female prevalence was evident. Patients presented most frequently with symptoms of hyperprolactinemia (44%) or visual disturbances (47%) when the sellar gangliocytoma was isolated, or with acromegalic manifestations (67%) when the gangliocytoma was associated with a pituitary adenoma. Immunohistochemistry showed a higher prevalence of mixed GH-PRL adenomas in association with sellar gangliocytomas, followed by GH adenomas. A purely intrasellar localization was found in about 25% of cases and a suprasellar extension in 30% of cases. A more extensive infiltration was present in about 40% of cases. A complete resection was obtained in 56% of collision lesions. The follow-up period was variable but endocrine remission was reported in 81% of cases of associated gangliocytoma and pituitary adenoma. Sellar gangliocytomas are rare lesions and an association with pituitary adenomas should always be searched. Mixed GH PRL adenomas are most frequently isolated. Collision lesions seem to have a behavior similar to isolated adenoma but a more important aggressiveness should be excluded at a molecular level. Further studies will allow advancements in clarifying the pathogenesis of this association.