A new case of pigmented paravenous retinochoroidal atrophy is described. Besides the characteristic fundus picture, this 21-year-old male showed a remarkable difference in retinal function between the two eyes, the function of the left eye being much more impaired. This might suggest a progressive condition, in which the right eye lags behind. In addition this patient had a vitreoretinal degeneration resulting in five peripheral retinal tears in the right eye.