Cystic retrochiasmatic craniopharyngioma may reach enormous size by expanding into the posterior fossa along the retroclival area, which is very unusual finding. An 11-year-old girl presented with walking disturbance and irritability. On the neurological examination, she had positive Babinski sign, hyperactive deep tendon reflex, and left lateral gaze nystagmus. She also had right hearing disturbance. Initial magnetic resonance(MR) images revealed a huge, thin capsuled cystic mass which extended into the cerebellopontine angle down to the medulla oblongata. Secondary adjuvant chemotherapy, radiotherapy, and reoperation were necessary after first tumor removal by a suboccipital approach, due to the progression of disease. Finally, neurological condition was much improved without remnant tumor although some sequelae were remained. We report this interesting case because of its rarity, complex clinical presentation, and challenge in its management.