Many patients with spinal tumours of developmental origin do not receive preoperative diagnosis and the surgical management, especially as for capsule resection, is often unplanned. Like other uncommon tumours, the intramedullary epidermoid cyst is often an operative or histological finding. Since magnetic resonance imaging (MRI) introduction, evidence has accumulated that they may be preoperatively suspected. In reporting the case of a young patient with a T3-T4 intramedullary epidermoid cyst, the authors present an overview of the clinical, radiological and surgical aspects of such tumors and review the latest literature in which MRI and microsurgical excision were performed. Despite the introduction of new diagnostic and surgical instrumentation, the preoperative diagnosis and surgical management of such tumours need further discussion.