Many spinal cord lesions have been described previously, in association with Chiari I lesions. The authors report a unique case of a 29-year-old patient with a Chiari I malformation and an upper thoracic epidural lesion causing headaches, dysphagia and Brown-Sequard syndrome. The patient underwent a suboccipital decompression as well as a thoracic laminectomy and resection of the epidural lesion. Pathologic analysis revealed that the thoracic lesion was fibro-adipose tissue. A review of the literature failed to show any other similar cases. We discuss the possible etiologies of this case.