Arachnoid cysts are benign, cerebrospinal fluid-filled collections that can be located in the brain or spinal cord. Arachnoid cysts form approximately 1% of all intracranial lesions. They are accepted as arachnoid developmental anomaly and arise from membrane splitting or duplication. Generally, lesion growth causes symptoms because of mass effect or obstruction. Arachnoid cyst growing mechanisms are a largely controversial issue. We report the case of a neonatal female patient who presented with congenital facial paralysis. Magnetic resonance imaging showed a right cerebellopontine angle arachnoid cyst causing severe mass effect on the brain stem. Cyst fenestration and cystocisternal shunt was performed through retrosigmoid suboccipital craniotomy.