RATIONALE: Craniopharyngiomas are histologically benign tumors with a relatively high recurrence rate. Surgical removal is challenging due to proximity to critical brain structures. This report introduces a staged operation strategy for a huge cystic craniopharyngioma. PATIENT CONCERNS: An 8-year-old girl presented with diplopia and decreased visual acuity. DIAGNOSES: Brain magnetic resonance imaging revealed a large, 10-cm-sized cystic mass encasing both the anterior and middle cerebral arteries and the optic nerve. INTERVENTIONS: An Ommaya reservoir was placed for periodic cyst aspirations over a period of 15 months. Subsequent magnetic resonance imaging indicated a reduction in cyst size, with an increasingly distinct tumor-pial interface evident on T2-weighted images. The tumor, initially entwined with neurovascular structures, gradually became delineated from these critical components. She underwent a secondary surgical intervention utilizing a bifrontal interhemispheric approach. Intraoperatively, the tumor was meticulously dissected and totally excised without compromising surrounding critical structures, while the tumor's origin, the pituitary stalk, underwent partial resection. OUTCOMES: There was no worsening of vision after surgery. She was on minirin medication, and there was no recurrence during the 1-year follow-up. LESSONS: Microsurgery of huge cystic craniopharyngioma following long-term cyst aspirations via Ommaya reservoir could present an efficacious strategy to diminish complication risks in pediatric patients.