Macular hole (MH) is uncommonly seen in association with retinitis pigmentosa (RP), optic disc pit or choroidal coloboma. The visual disability in eyes with RP, optic disc pit or choroidal coloboma can range from mild to severe. However, the macula may not be involved in the primary pathology and hence, the central vision may be preserved in these eyes. If a MH develops in such eyes the central vision is lost, making the patient severely vision disabled. A perceived poor prognosis in such patients may lead to no intervention. However, an attempt should be made to repair and restore the macula in these situations in order to conserve the vision and limit disability. These eyes can however pose unique surgical challenges. We report our experiences of operating on cases of MH associated with unusual concomitant pathologies such as RP, choroidal coloboma, and optic pit. The outcomes and the surgical challenges differed greatly from the conventional idiopathic MH surgery.