The human retina is amenable to direct, noninvasive visualization using a wide array of imaging modalities. In the ∼140 years since the publication of the first image of the living human retina, there has been a continued evolution of retinal imaging technology. Advances in image acquisition and processing speed now allow real-time visualization of retinal structure, which has revolutionized the diagnosis and management of eye disease. Enormous advances have come in image resolution, with adaptive optics (AO)-based systems capable of imaging the retina with single-cell resolution. In addition, newer functional imaging techniques provide the ability to assess function with exquisite spatial and temporal resolution. These imaging advances have had an especially profound impact on the field of inherited retinal disease research. Here we will review some of the advances and applications of AO retinal imaging in patients with inherited retinal disease.