Hypertrophic cardiomyopathy (HC) may transition to a phase characterized by systolic impairment resembling dilated cardiomyopathy. This study retrospectively assessed the incidence of left ventricular (LV) systolic impairment at initial clinical evaluation in 248 consecutive patients with HC (mean age 53 ± 16 years). HC with systolic impairment was diagnosed if the LV ejection fraction was <50%, calculated by echocardiography. Twenty patients (8%) had HC with LV systolic impairment at initial evaluation. Patients with systolic impairment had a greater incidence of family histories of sudden cardiac death (SCD) than patients with preserved systolic function (25% vs 5.3%, p = 0.006) and more severe functional limitations (New York Heart Association class ≥III, p <0.001). All-cause mortality and cardiovascular mortality did not differ between the 2 groups. The incidence of SCD was 1.7% in patients with normal LV ejection fractions, and no SCD was observed in patients with systolic impairment. The latter group had more frequent major cardiac events (SCD, ventricular fibrillation, aborted cardiac arrest, and first implantable cardioverter-defibrillator discharge; p = 0.03). During follow-up, 2 patients progressed to HC with systolic impairment (annual incidence 0.85%). In conclusion, systolic impairment is not exceptional in patients with HC at initial evaluation and is associated with functional deterioration and major cardiac events.