Intramedullary epidermoid cysts are extremely rare lesions. Preoperative diagnosis can be challenging due to non-specific imaging futures. We illustrate the case of a 32-year-old man who presented with a symptomatic intramedullary mass, which at surgery proved to be an epidermoid cyst. The spinal cord was decompressed by removing the pearl-white keratin material, although the capsule, which was tenaciously adherent to the surrounding spinal cord parenchyma, was only partially removed. In this video, we illustrate the salient surgical points for the resection of this very unusual intramedullary lesion.