OBJECTIVE To describe a case of Goldmann-Favre vitreoretinal degeneration with typical clinical findings. METHODS The case report of a healthy 47-year-old woman with typical clinical findings of Goldmann-Favre vitreoretinal degeneration is presented. She had complaints of reduced visual acuity and night blindness. Her parents were first cousins. RESULTS Fundus examination revealed annular pigmentary degenerative changes, macular edema, and peripheral retinoschisis in both eyes. Electroretinography results were abnormal. Optical coherence tomography scans revealed hyporeflective spaces in the macular area and irregularities of the chorioretinal complex in the degenerative pigmentary areas. CONCLUSIONS Typical fundus findings combined with night blindness and electroretinogram abnormalities permited the diagnosis of Goldmann-Favre vitreoretinal degeneration.