Body image disturbance is an important feature of Anorexia Nervosa (AN). Some researchers have argued that body image disturbance is not just a symptom of AN, but plays a causal role in the development, persistence, and relapse of AN. Our aim was to systematically review the existing empirical evidence concerning the role of the cognitive-affective, perceptual, and behavioral components of body image disturbance in AN. 46 studies fulfilled eligibility criteria reporting about 4928 participants with AN. There is some evidence suggesting that body image disturbance is related to the course of AN. However, experimental studies were missing and operationalizations of body image constructs and AN outcome measures varied greatly across studies. Therefore, on the basis of the available empirical data, it remained unclear whether body image disturbance is indeed a causal risk factor for AN. For future studies, it is crucial to use more consistent terminology and more specific and precise definitions of body image constructs as well as experimental designs, adequately powered samples, and well-validated measures. Altogether, this would set the stage to generate the high-quality data that are necessary to clarify the role of body image disturbance in the onset, maintenance and relapse of AN.