Recent diagnostic and therapeutic advances have provided new hope for patients with inherited retinal degenerations and have brought a previously obscure field to the forefront of medical scientific advances. As compared to 1989, when only 1 genetic mutation was known to cause an inherited retinal disease, several thousand mutations in more than 190 genes are now recognized. With each year, our understanding of these diseases, our diagnostic tools, and our molecularly based treatments continue to expand, making it challenging to keep abreast of these advances. This module provides an overview of the genetics of selected retinal degenerative diseases, highlighting pertinent clinical diagnostic features and relevant gene-based therapies.