The case concerns a 54-year-old woman with macular choroidal coloboma (congenital developmental disorder) who had reduced visual acuity of 0.05 in the right eye and metamorphopsia (distorted vision). Diagnostics using optical coherence tomography revealed a macular hole of size 1238 × 976 μm together with a posterior scleral staphyloma (arching of the eye wall). The patient underwent vitrectomy (surgical removal of the vitreous) with amniotic membrane transplantation, peeling of the internal limiting membrane and silicone oil tamponade. At the 3-month follow-up, the amniotic membrane was functional, but the condition was not fully stabilized, so a second amniotic membrane transplantation was performed during silicone oil removal. Nine months after surgery, optical coherence tomography showed firm adhesion of the amniotic membrane with the edges of the macular hole and reduction of the cavity created by the coloboma. In parallel with the anatomical improvement, the patient's visual acuity also improved. This case demonstrates that surgical treatment led to improvement of ocular anatomy and visual quality in this rare disease.