Acute presentation of cystic craniopharyngioma is a potentially life-threatening condition, characterized by disability and increased mortality risk, often caused by large cysts with or without hydrocephalus. This study evaluated the applicability of minimally invasive neuroendoscopic surgery (NES) for rapid intracranial pressure relief and tumor control as an alternative to emergent microsurgical resection. A retrospective review of the electronic medical record database of patients with craniopharyngiomas treated at our institution between June 2007 and October 2024 identified 13 non-consecutive cases of acute presentation of cystic craniopharyngioma managed with NES. Interventions included pellucid septostomy (n = 2), ventriculoperitoneal shunt surgery (n = 2), and Ommaya reservoir placement (n = 6). Clinical and radiological data were analyzed to assess treatment outcomes. The most common acute symptoms were severe headache (69.2%), frequent vomiting (53.8%), sudden onset of consciousness disturbance (38.5%), visual impairment (46.2%), gait instability (23.1%), and cognitive dysfunction (15.4%). Ten patients had hydrocephalus at admission. All patients experienced symptom relief following NES, with no procedure-related mortality or complications. Hydrocephalus resolved in all cases, and subjective visual function improved in 83.3% of patients postoperatively. Endocrine function remained stable. At a median follow-up of 62 months (range, 38-130), local tumor control was achieved in 8 patients (61.5%) without requiring adjuvant therapy. NES is a safe, effective approach for managing acute presentation of cystic craniopharyngioma, particularly in patients with hydrocephalus. It offers rapid symptom relief and tumor control and is a potential staging treatment in selected cases.