Body image is the way a person's body feels to them. Growing evidence shows that body image can be distorted in people with pain, particularly chronic pain. Most data relate to people with deafferentation via amputation or neural injury, but deafferentation is neither sufficient nor necessary for distorted body image or pain. In this review, we examine the literature relating to body image distortion in people with pain, and we discuss three themes: 1) evidence of distorted body image in people with pain; 2) evidence of distortion of the neural representations of body image held in primary sensory and primary motor cortex; and 3) clinical findings that correlate with distorted body image, distorted neural representation, or both. We then review the emerging evidence regarding therapeutic approaches to distorted body image in people with painful disease.