Choroidal colobomas represent failure of closure of the fetal fissure during embryogenesis. Eyes with colobomas can develop complex retinal detachments. We present a case of a patient presenting with a macular hole in her only useful eye with an extramacular choroidal coloboma.A 56-year-old woman was referred with a 3-month history of central distortion and blurring of vision in her right eye. The left eye was amblyopic with microphthalmos. She was found to have a Stage 2 macular hole in her right eye with a large inferonasal coloboma extending underneath the inferior arcade. The patient underwent vitrectomy with induction of a posterior vitreous detachment, internal limiting membrane peeling, and insertion of C2F6 gas.During the vitrectomy, there was some difficulty in inducing a posterior vitreous detachment along the edges of the coloboma, but otherwise, the stronger vitreous adhesion did not interfere with the procedure. The patient responded well to surgery, and the macular hole closed.The occurrence of a macular hole in an eye with choroidal coloboma has not been previously described. The presence of a coloboma causes uncertainty about performing a vitrectomy, because colobomatous eyes are at risk of retinal detachment. This case demonstrates that vitrectomy and induction of a posterior vitreous detachment does not necessarily lead to retinal detachments in such cases, and successful management of a condition like macular holes is possible in the presence of retinal coloboma.