We describe the presentation and management of a rare case of an epidermoid cyst located within canal of the left internal acoustic meatus, lying entirely within the petrous part of the temporal bone. The patient underwent surgery by an extended middle fossa approach and a total excision of a cyst containing cheesy, pearly white, avascular material was achieved. Neuroradiology provides a diagnosis with reasonable accuracy and an appropriate surgical approach can be planned for the excision of the tumour.