The presence of syncope despite medical therapy in patients with hypertrophic cardiomyopathy (HC) is considered an indication for surgical myectomy; however, no study has examined the long-term effects on recurrent syncope and survival after surgery in these patients. We examined 239 patients with HC and a history of syncope who had undergone surgical myectomy (mean age 48 ± 17 years; 56% men). The patients were age- and gender-matched to patients with HC and syncope who were treated medically without myectomy (mean age 51 ± 16 years; 59% men). The median follow-up period was 4.7 years (0.8, 11.3). The recurrence rate of syncope was 11% in the myectomy patients and 40% in the medical group (p <0.0001). Multiple episodes of syncope, left ventricular outflow tract obstruction, and recent syncope were identified as baseline predictors of recurrent syncope. Survival free of all-cause mortality was greater for patients who had undergone surgical myectomy than for the medically treated patients (10-year estimate 82 ± 4% vs 69 ± 4%; p = 0.01). In conclusion, surgical myectomy in patients with HC and a history of syncope was associated with a reduction in recurrent syncope and increased survival.