PURPOSE OF REVIEW: Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiomyopathy, diagnosed by left ventricular hypertrophy of at least 15 mm maximal wall thickness (MWT). Recent studies reported a sex difference in clinical presentation, progression and outcome of HCM. This review provides an overview of recent studies into sex differences in HCM. RECENT FINDINGS: A higher number of men (55-65% of total HCM patient group) with manifest HCM has been observed, whereas female patients are older at first evaluation and diagnosis, present more frequently with symptoms, and have worse survival. Additionally, women have relatively smaller hearts even when corrected for body surface area (BSA), but female HCM patients have a higher interventricular septum thickness after correction for BSA. SUMMARY: Female HCM patients are possibly in a more advanced stage of disease at time of diagnosis because they require relatively more hypertrophy to reach the diagnostic threshold of at least 15 mm MWT. Additional studies are warranted to explore sex-specific diagnostic criteria for HCM.