Background: Organic tricuspid valve (TV) disease is uncommon. Few studies have compared TV repair with replacement in these patients. The current study compared midterm outcomes of TV repair versus replacement in a large group of patients with organic tricuspid disease. Methods: Two-hundred and fifty patients underwent surgery for organic TV disease at our institution from 1979 to 2003. Clinical and echocardiographic follow-up were obtained, were 99% complete, and were 5.2 ± 4.1 years long (mean ± standard deviation). Results: One hundred and seventy-eight patients (71%) underwent TV repair and 72 (29%) received TV replacement (54 bioprosthetic, 18 mechanical). Repair patients were more likely to have hypertension, rheumatic pathology, or elective surgery. Concomitant procedures included mitral (50% of patients), aortic (26%), and coronary bypass (6%) operations. Perioperative and midterm mortality were higher in the replacement group (both p < 0.001). Cox regression analysis revealed TV replacement as an independent predictor of midterm mortality (hazard ratio: 5.1, 95% confidence interval: 2.9 to 9.1, p < 0.001) and decreased event-free survival (hazard ratio: 2.0, 95% confidence interval: 1.1 to 3.6, p = 0.02). Follow-up echocardiography revealed more moderate to severe tricuspid regurgitation in repair patients (38% vs 5%, p < 0.001), but no difference in New York Heart Association functional class or reoperation rates. Conclusions: Tricuspid valve repair is associated with better perioperative, midterm, and event-free survival than TV replacement in patients with organic tricuspid disease. Despite more tricuspid regurgitation in the repair group during follow-up, reoperation rates and functional class were similar. Repair should be performed whenever possible in patients with organic tricuspid disease.