Intramedullary epidermoid cysts of the spinal cord are rare lesions. The authors report a case of giant intramedullary epidermoid cyst at the cervicothoracic region. A 6-year-old boy presented with motor and sensory loss related to a spinal intramedullary epidermoid cyst. Almost all cyst content was liquid and it was surgically removed totally in two successive operations. To the author's knowledge, this is the largest intramedullary epidermoid cyst published in the literature and no other case of intramedullary epidermoid cyst with a complete liquid content has been reported before. The authors discuss the clinical features, MR imaging characteristics and surgical findings of this rare tumor and review the associated literature. Copyright (C) 2004 S. Karger AG, Basel.