Background and Purpose: Executive functions are very important in achieving the educational and social success in childhood. This term includes a set of cognitive abilities like planning, attention control, working memory, inhibition, and cognitive flexibility. Nowadays, the theoretical models emphasize on the role of visual limitation in the development and evaluation of executive functions. Therefore, the purpose of this study was to systemically review the development and pathology of executive functions in children with visual impairment. Method: This study was conducted as a systematic review; in such a way that PupMed, Springer, Scopus, ProQuest, Science direct, and Google Scholar databases were searched for the keywords of executive functions, blindness, congenital blindness, visual impairment, cognitive abilities, memory, working memory, auditory memory, inhibition, attention, and short-term memory to obtain the research results from 1995 to 2019. Finally, among the 49 articles searched, 21 articles were included in our research based on the inclusion criteria and PRISMA Checklist. Then the results were classified, summarized, and reported. Results: Evaluation of the articles showed that in spite of the inevitable impact of visual sense in the development of executive functions, factors like age at onset of blindness, sensory compensation, spatial-auditory attention and auditory processing, orientation and mobility skills, cerebral cortex flexibility, changes in the brain structure, and the intelligence function play an important role in the normal development of executive functions of children with visual impairment. Conclusion: The results showed that the development of executive functions in children with visual impairment has the same complexities of executive functions in normal children and cannot be explained by a single factor. However, personal and environmental experiences play a greater role in the development of visually impaired children's executive functions. Therefore, paying attention to these factors can help us to achieve a better understanding of the executive functions in these children and design new educational, rehabilitation and interventional approaches.