Posterior microphthalmos is a rare ocular disorder characterized by disproportionately short vitreous length and numerous posterior segment findings. We report a rare coexistence of bilateral macular hole with posterior microphthalmos in a 15-year-old girl. Detailed ophthalmic examination and investigations including B scan ultrasonography and optical coherence tomography were performed. Ophthalmic examination showed bilateral high hyperopia. Best corrected visual acuity was 6/18 in each eye with correction of +12 D in right eye and +10 D in left eye. Ocular biometry measurements showed short total axial length (16.3 mm both eyes) with remarkable shortening of posterior segment (9.3 mm right eye; 9.28 mm left eye) but normal anterior segment parameters. Anterior chamber depth and horizontal corneal diameter were within normal range. Fundus evaluation revealed bilateral macular hole. Optical coherence tomography finding was compatible with bilateral lamellar macular hole in both eyes. B scan ultrasonography showed sclerochoroidal thickening with foreshortening of vitreous cavity. A variety of posterior segment manifestations associated with posterior microphthalmos have been reported but macular hole is an extremely rare association. This finding is noteworthy and probably among very few in literature.