A 22-year-old woman presented with a rare case of multiple epidermoid cysts located in the pineal and extracranial regions. Magnetic resonance (MR) imaging showed a lesion in the pineal region as hypointense on the T(1)-weighted image and hyperintense on the T(2)-weighted image, without enhancement. Neuroendoscopic treatment was performed under a diagnosis of pineal cyst. However, the cyst wall was too thick to perforate, although third ventriculostomy was performed. Diffusion-weighted MR imaging demonstrated the lesions in the pineal and extracranial regions as marked hyperintensity. The diagnosis was epidermoid cyst. Subsequently, neuroendoscopic treatment of the pineal epidermoid cyst was performed. Careful preoperative diagnosis of epidermoid cysts based on diffusion-weighted MR imaging is required.