Supplemental Digital Content is Available in the Text. NPHP1-associated retinal degeneration can present as a cone–rod dystrophy with relative foveal preservation. This phenotype is increasingly recognized in other oculorenal ciliopathies, such as Bardet–Biedl and Alström syndromes, suggesting a common or intersecting mechanism of disease. This report also supports that there may be a consistent NPHP1-retinal phenotype rather than diversity.