PURPOSE: To describe the clinical features and management of the largest reported series to date of patients with diffuse unilateral subacute neuroretinitis (DUSN). DESIGN: Observational case series. METHODS: The charts of all patients coded as having DUSN in a vitreoretinal clinic in Caracas, Venezuela between July 1979 and August 2000 were retrospectively reviewed. MAIN OUTCOME MEASURES: Demographic information, presenting visual acuity, clinical course, and treatment were evaluated. RESULTS: We identified 82 eyes of 78 patients with DUSN. The mean age at diagnosis was 16.7 years, significantly younger than the mean age of the Venezuelan population. Sixty-five (83.3%) patients were 20 years old or younger (P<0.001 when compared with the population of Venezuela). Thirty-three (42.3%) of the patients were female. The presenting visual acuity was 20/400 or worse in 69 eyes (84.1%). The subretinal nematode was identified in 33 eyes (40.2%). All nematodes were small, approximately 400 microm in length. CONCLUSIONS: In Venezuela, DUSN is seen primarily in young patients without a significant gender predilection. The vast majority of patients in this population presented with severe vision loss that was irreversible.