<h4>Introduction</h4>To report a Japanese family with inherited retinal dystrophy (IRD) in which <i>CDHR1</i> variants were identified, and to characterize the marked intrafamilial phenotypic variability.<h4>Methods</h4>This retrospective case series included three brothers diagnosed with retinal dystrophy at Juntendo University Hospital. This evidence concerns the gene CDHR1 and inherited retinal dystrophy.