Purpose: To report vitrectomy with the inverted internal limiting membrane (ILM) flap technique in a patient with a full-thickness macular hole (FTMH) and Coats disease. Methods: A case and its long-term findings were analyzed. Results: A 27-year-old patient with Coats disease who was treated 5 years earlier with laser photocoagulation presented with an FTMH. Vitrectomy with the temporal inverted ILM flap technique was performed. The macular hole decreased in size on serial OCT scans but did completely close until 18 months postoperatively. The final visual acuity was 20/40 (0.3 logMAR). The patient’s vision remained stable for the next 5 years. Conclusions: Although the healing process after vitrectomy with ILM peeling and the inverted flap technique in an FTMH coexisting with Coats disease is prolonged compared with an idiopathic FTMH, it is still possible to obtain satisfactory anatomic and functional results.