Two types of Usher syndrome, a blindness–deafness disorder, result from mutations in the myosin VIIa gene. As for most other unconventional myosins, little is known about the function or functions of myosin VIIa. Here, we studied the photoreceptor cells of mice with mutant myosin VIIa by electron immunomicroscopy and microscopic autoradiography. We found evidence that myosin VIIa functions in the connecting cilium of each photoreceptor cell and participates in the transport of opsin through this structure. These findings provide the first direct evidence that opsin travels along the connecting cilium en route to the outer segment. They demonstrate that a myosin may function in a cilium and suggest that abnormal opsin transport might contribute to blindness in Usher syndrome.