Purpose This study aimed to evaluate macular perfusion using optical coherence tomography angiography (OCTA) in patients with cone dystrophy and to determine the associations between the quantitative data of OCTA and functional parameters. Methods The data of 36 eyes of 18 patients with cone dystrophy and 38 eyes of 19 healthy controls were analyzed. The superficial and deep capillary plexus (SCP and DCP) vessel densities (VD) of the macula, the foveal avascular zone (FAZ) and choriocapillary flow density values were obtained using OCTA. The associations between visual acuity (VA) and full-field electroretinography (ffERG) and the quantitative data of OCTA, and the associations between OCTA and ffERG were analyzed. Results : VD was significantly lower in all areas except the foveal area in SCP in the cone dystrophy group compared to the control group. VA was found to be associated with the VDs of the SCP and DCP except for that of the foveal SCP. VA was also associated with dark-adapted, light-adapted wave amplitudes. Conclusion OCTA quantitatively showed that macular perfusion was decreased in cone dystrophy compared to the healthy controls. In addition, there was an association between VA and ffERG parameters and quantitative data of OCTA.