Purpose. The aim of the study was evaluation of the scientific evidence about the efficacy of vision therapy in children and teenagers with anisometropic amblyopia by performing a systematic literature review. Methods. A search was performed using 3 searching strategies in 4 different databases (PubMed, Web of Science, Scopus, and PruQuest). The quality of the included articles was evaluated using two tools for the risk of bias assessment, ROBINS-I for nonrandomized studies of intervention (NRSI), and ROB 2.0 for randomized clinical trials. Results. The search showed 1274 references, but only 8 of them passed the inclusion criteria after the complete text review. The articles that were finally included comprised 2 randomized control trials and 6 nonrandomized studies of intervention. These articles provided evidence supporting the efficacy of vision therapy for the treatment of anisometropic amblyopia in children and teenagers. Assessment of the risk of bias showed an appropriate risk of bias for the randomized control trials, but a high risk of bias for nonrandomized studies of intervention (NRSI). A main source of risk of bias for NRSI was the domain related to the measurements of the outcomes, due to a lack of double-blind studies. Conclusion. Vision therapy is a promising option for the treatment of anisometropic amblyopia in children and teenagers. However, the level of scientific evidence provided by the studies revised is still limited, and further randomized clinical trials are necessary to confirm the results provided to date and to optimize the vision therapy techniques by knowing the specific neural mechanisms involved.