BACKGROUND: We report a rare case of arachnoid cyst incorporating choroid plexus. This 7-month-old girl presented with macrocrania. Magnetic resonance (MR) imaging disclosed a cystic lesion arising from the left prepontine cistern extending to the left middle cranial fossa. METHODS: First, we performed resection of the membrane microscopically and obtained an adequate reduction of the cystic size. However, 4 months after the first operation, the cyst was enlarged again, and bulging of the portion of the craniotomy was noted. Thus, we performed neuroendoscopic transcortical ventriculo-cyst-cysternostomy and confirmed the choroid plexus inside of the arachnoid cyst. DISCUSSION: Postoperative course was uneventful. This is the first reported case of choroid plexus within an arachnoid cyst, determined neuroendoscopically.