Syringomyelia is not a single disease, but rather a descriptive term for any fluid cavity in the spinal cord. Regardless of the underlying etiology, the signs and symptoms of syringomyelia are related to the location, size, and extent of the cavity. With the advent of magnetic resonance imaging (MRI) and improved surgical procedures for decompression of the cavity, syringomyelia is an increasingly recognized cause of disability and even death in patients with clinical signs and symptoms of central spinal cord lesions. In this review, the etiology, pathophysiology, imaging, and treatment will be concisely discussed.