Purpose: To report a case of Best vitelliform macular dystrophy complicated by macular hole. Methods: History and clinical examination, electroretinogram, and optical coherence tomography. Results: We report a case of a 20-year-old boy with progressive visual loss in his left eye. In fundus examination, there was a typical vitelliform lesion in the right eye and a macular hole in the left eye. The electroretinogram Arden ratio was ,1.1 in both eyes. Optical coherence tomography revealed deposition of hyperreflective material and hyporeflective area between the junction of the inner segment and outer segment of the photoreceptors and the retinal pigment epithelium in the right eye, and large full-thickness macular hole with cystoid changes in the retinal layers in the left eye. There was no associated retinal detachment. Conclusion: Although extremely rare, macular hole should be considered as a cause of the significant visual loss in patients with Best vitelliform macular dystrophy.