PURPOSE: Macular telangiectasia type 2 (MacTel2) is a rare disorder, affecting retina's vascular structure, and MacTel2-associated full-thickness macular holes (MHs) are extremely rare in general population. Since their management is still controversial, this literature review was aimed to gather evidences on the surgical management of MacTel2-associated MHs. METHODS: A retrospective review on the Cochrane Central, PubMed, Web of Science, and ClinicalTrials.gov databases for articles regarding MacTel2 and MHs. The search period was set from January 2000 to February 2024, and any kind of article was taken into account. RESULTS: We took into account 10 studies analyzing surgical outcomes of MacTel2-associated MHs, with a total number of 95 treated eyes. Different surgical techniques were used, with a better anatomical outcome after internal limiting membrane inverted flap surgery but with very controversial functional outcomes. In a comparative report, internal limiting membrane peeling alone (34 eyes) was associated with a 47% anatomical success rate, internal limiting membrane inverted flap (22 eyes) with 90% successful closure, and autologous retinal transplantation (five eyes) with a 100% success rate but without visual improvement. CONCLUSION: Since the exact pathophysiology of MacTel2 macular holes is not clear yet, even if the internal limiting membrane inverted flap technique could be an effective treatment for these patients, visual outcomes are poor. This underlines the necessity of a deeper comprehension of the pathophysiology of the disease and the development of new surgical approaches.