Progressive cone dystrophies represent a heterogeneous group of diseases with onset in later teens and adult age diagnosed by an abnormal photopic electroretinographic response and a normal or near-normal rod isolated response. We report the case of a young male with typical characteristics of cone dystrophy and a brief description about the inheritance pattern and pathology of the disease.Progressive cone dystrophies represent a heterogeneous group of diseases with onset in later teens and adult age diagnosed by an abnormal photopic electroretinographic response and a normal or near-normal rod isolated response. We report the case of a young male with typical characteristics of cone dystrophy and a brief description about the inheritance pattern and pathology of the disease.