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Nutritional status has been related to clinical outcomes in patients with cardiovascular diseases. The prognostic impact of poor nutritional status in hypertrophic cardiomyopathy (HCM) is not clearly understood. The aim of the present study is to investigate the prognostic value of prognostic nutritional index (PNI), calculated from serum albumin level and total lymphocyte count, in HCM patients. A total of 393 HCM patients in a tertiary medical centre were enrolled. The primary and secondary endpoints were all-cause mortality and cardiovascular death. The association between PNI and endpoints was analysed. During a mean follow-up duration of 4.8 years, patients with high PNI values (PNI ≥ 48.8) had significantly lower incidence of all-cause mortality (9.3% vs. 33.1%, P < 0.001) and cardiovascular death (7.1% vs. 21.0%, P < 0.001). After adjusting for potential confounders, PNI was independently associated with all-cause mortality and cardiovascular death (hazard ratio per 1 SD increase: 0.46 [95% CI: 0.34–0.62, P < 0.001] and 0.44 [95% CI: 0.30–0.63, P < 0.001]). In subgroup analysis stratified by age, gender, New York Heart Association class, atrial fibrillation, estimated glomerular filtration rate, left ventricular ejection fraction or left ventricular outflow tract obstruction, PNI was consistently related to mortality. PNI is an independent prognostic factor for mortality in patients with HCM. • Prognostic nutritional index (PNI) reflects individual's immunonutritional status. • It has been regarded as a useful indicator for poor prognosis in several diseases. • The prognostic value of PNI in HCM patients was initially assessed. • PNI significantly predicts all-cause mortality and cardiovascular death in HCM. • The best cut-off value of PNI was 48.8 for our HCM population. [ABSTRACT FROM AUTHOR]