Nonenhancing Prepontine Chordoma with Diffusion Restriction Mimicking an Epidermoid Cyst

Intradural prepontine chordoma is an extremely rare entity and depicts benign features with no recurrence and has a good prognosis as compared with bone endogenous chordoma. Radiologically, it may mimic an epidermoid cyst and present an imaging challenge. Here we present a case of a middle adolescent girl who presented with headache along with limb and facial weakness. A nonenhancing, diffusion-restricting, predominantly intradural cystic-appearing mass lesion was seen in the prepontine cistern. Initially, an epidermoid cyst was suspected, but histopathology revealed it to be a chordoma. Destruction of the clivus is important and chordoma should be included in differential diagnosis.