PurposeThis study aimed at assessing the macular anatomical and functional changes in patients having different stages of best vitelliform macular dystrophy (BVMD).DesignThis is a retrospective noncomparative case series.Materials and methodsThe study included patients in different stages of BVMD. All patients had a solitary lesion involving the fovea. They underwent complete ophthalmic evaluation, fluorescein angiography (FA), optical coherence tomography (OCT), and multifocal electroretinography (mfERG). The results were compared with those of an age-matched and sex-matched control group.ResultsThis study included 14 eyes from 10 patients belonging to seven families. The mean log MAR BCVA was 0.5 ± 0.1 with significant reduction compared with the control group (P = 0.05). Both FA and OCT scans showed changes confined to the lesion only. OCT showed a significant increase in thickness in the foveal and perifoveal rings (P = 0.0003 and 0.05, respectively). Multifocal ERG showed significant changes between the study and control groups in the three rings (P = 0.0001 for both amplitude and implicit time for ring 1; P = 0.05 for the other two rings).ConclusionIn different stages of BVMD with solitary lesions involving the fovea, the integrated FA and OCT findings were very helpful in the diagnosis; however, their changes were confined to the lesions only. Multifocal ERG revealed reduction of the cone function all over the macula, which was most evident centrally.