Background: Binder syndrome is a rare congenital malformation of the midface. Since 1990, no systematic review has been conducted on this condition. Our study aims to review and collect the surgical treatment options available for correcting nasomaxillary dysplasia and the information available in the international literature on Binder syndrome and associated symptoms. Methods: This systematic literature review was registered in the PROSPERO (International Prospective Register of Systematic Reviews) database. Systematic literature searches were performed in Medline, Scopus, the Cochrane Library, and Web of Science databases. Study quality assessment was performed according to recommendations from the Cochrane Handbook for Systematic Reviews of Diagnostic Test Accuracy. Results: The authors identified 200 articles from our database research. One hundred sixty-nine articles were excluded after the title and abstract screening. Seven articles were excluded after screening the full text. In total, 24 clinical studies were included: 14 studies aim to describe a surgical option and 10 articles treat information about Binder syndrome or associated features. Most of the studies (12/14 studies) performed rhinoplasties to treat nasomaxillary dysplasia. The 2 other articles evaluated maxillary osteotomy techniques. Only 3 articles compared the results obtained from different surgical techniques. Conclusions: No optimal surgical treatment plan has been developed, even if numerous surgical options are available. Etiology is still uncertain.