We report the cases of a father and his daughter, the former diagnosed with retinitis pigmentosa (RP) and the latter with early foveal atrophy; while both shared a novel variant of uncertain significance (VUS) in the <i>ACBD5</i> gene (variant c.431G>A), they exhibited different clinical profiles and disease manifestations. The gene discussed is ACBD5; the disease is Atrophy.