This report presents a rare presentation of a ganglioglioma in the sellar/suprasellar region. On the basis of the patient's presentation and imaging characteristics, the initial diagnosis was craniopharyngioma. While gangliogliomas are already rare brain tumors that are usually found in the frontal and temporal lobes of young patients, the presentation of this tumor in the sellar region is exceedingly rare.A 25-year-old male presented to the emergency department with headache, agitation, and combativeness. A head computed tomography scan showed a sellar/suprasellar mass with mixed solid and cystic components and peripheral calcifications. The mass compressed the third ventricle and cerebral aqueduct, resulting in obstructive hydrocephalus. The patient was intubated for decline in mental status and combativeness. A ventricular drain was placed emergently. A pituitary function panel did not show endocrine dysfunction. Magnetic resonance imaging showed a 3.6 cm × 4.2 cm solid mass in the sellar/suprasellar region with a cystic component. The mass displaced the adenohypophysis and extended into the prepontine and interpedicular cisterns. The clinical presentation and radiologic characteristics led to an initial diagnosis of craniopharyngioma. The patient underwent a right pterional craniotomy and transsylvian approach for resection of mass without complication, although a subtotal resection was achieved due to adherence of the tumor to optic nerves and carotid arteries. The resected specimen was diagnosed as ganglioglioma.This case is a reminder of how much the field of neurosurgery relies on imaging modalities but also emphasizes the importance of histopathology in the field of brain tumors.