OBJECTIVE: Mixed gangliocytoma-pituitary adenomas (MGPAs) are very rare sellar neoplasms. Contemporary understanding of their natural history, clinical characteristics, optimal management strategies, and prototypical outcomes remains poorly understood. METHODS: Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, PubMed, Embase, Web of Science, and Cochrane were searched to include studies of patients with histologically confirmed mixed gangliocytomas. Clinical features, management strategies, and outcomes were analyzed. RESULTS: Thirty three studies reporting 78 patients met study criteria and were included. Median age at diagnosis was 47 years, with a female predominance (71%). Common presenting symptoms included headache (49%), acromegaly (43%), and visual disturbance (20%). Growth hormone and prolactin were the most commonly elevated hormones. Magnetic resonance imaging identified local infiltration in 86% of patients, most frequently in the cavernous sinus. Transsphenoidal resection was the primary treatment strategy, which achieved a gross total resection in 43%. Postoperative pituitary function was normal in 92%. Median follow-up was 21 months, during which time 14% of patients required secondary intervention for persistent hormonal hypersecretion. Following these secondary interventions and as of last follow-up, radiographic and/or biochemical control was confirmed in all patients, with an overall survival rate of 97%. CONCLUSIONS: MGPAs are rare, slow-growing tumors that present with a combination of endocrinological and neurological symptoms. MGPAs are optimally managed with transsphenoidal resection, which appears to achieve favorable rates of symptomatic relief and local control despite the high incidence of local infiltration and relatively low attendant gross total resection rate.