Risk stratification and primary prevention of sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM) is a challenging discipline. When using current stratification systems, there is always a compromise between sensitivity and specificity. In our pilot study, we sought to determine the incidence of SCD and the effectiveness of stratification systems, i.e., the ESC HCM Risk-SCD score and the system of individual major risk factors (RF) according to ACC/AHA in patients followed in an expert center. Patients with HCM were risk-stratified and followed for 10 ± 8 years at the referral HCM center. Results: Five hundred fifty-six patients were stratified, and 71 (13%) were implanted with a cardioverter-defibrillator (ICD) based on risk stratification. During follow-up, 12 (17%) patients had ≥ 1 appropriate ICD therapy. None of the ICD patients died of SCD. During follow-up, 118 (21%) patients died, of which 28 (5%) from HCM-related causes, 12 (2%) from stroke, 11 (2%) from advanced heart failure, one perioperatively (myectomy) and 4 (1%) patients died suddenly (SCD). In conclusion, with current risk stratification, albeit imperfect, SCD is rare in HCM patients managed at an expert center.