Gangliogliomas are usually benign slow-growing neoplasms, seen mainly in the first 3 decades of life and are prevalently located supratentorial, mostly in the temporal and frontal lobe. The authors present a rare case of a brainstem ganglioglioma in an 11-year-old boy who was referred to their hospital complaining of episodes of blurry vision, loss of memory, gait disturbances, and morning headache with vomiting, lasting for over a month. Computed tomography and magnetic resonance imaging scans revealed a mass on the dorsal surface of the brainstem, compressing the brainstem and producing secondary obstructive hydrocephalus. The patient was operated upon, and the histopathology revealed the presence of a ganglioglioma grade II (World Health Organization classification). On follow-up examination after 1 year, a minor gait imbalance was the only finding. A total resection should be always attempted, where possible in brainstem gangliogliomas. Close follow-up is mandatory, and re-resection or radiotherapy should be considered in case of tumor recurrence.