We report an unusual case of epidermoid cyst located in the cavernous sinus. Only two cases of epidermoid cyst of the cavernous sinus have been reported in the literature. In 1995, a 61-year-old man experienced diplopia, which resolved spontaneously. Imaging findings, particularly magnetic resonance, showed a cavernous sinus tumor. The MR appearance was compatible with epidermoid cyst. Since the patient was, at the time, asymptomatic, we proposed clinical and MR surveillance. In 1999, he developed a right III palsy with V1 and V2 hypoesthesia. The size of the cavernous tumor had increased on the control MR and the patient was operated via an extradural temporopterional approach through the Dolenc lateral triangle. We used a neuroendoscope to perform total resection of the tumor cyst. The extradural approach to the cavernous sinus and use of a neuroendoscope allowed complete removal of the cyst and shortened postoperative care. The patient was discharged on day 5 after surgery. These techniques provided a good view of the tumor without orbitozygomatic or zygomatic osteotomy. The 30 neuroendoscope allowed visual control of tumor removal that was better than a direct microscope view.