Epidermoid cyst may rarely arise within the lateral wall of the cavernous sinus (CS) and produce clinical symptoms mimicking the Tolosa-Hunt syndrome. Pathogenically, it is suggested that some of the subpopulation of cells arising from the neural crest which give rise to skin, may remain entrapped in the meninges around the nerves in the lateral wall in an uncompleted stage of maturation and may develop epidermoid cysts. A case is presented of a 68-year-old man with a 4-week history previously diagnosed as Tolosa-Hunt syndrome. MRI is the investigation of choice. Surgical resection can result in excellent recovery of preoperative deficits of cranial nerves (CN) III through VI.