We report the case of a 45 year-old woman with systemic lupus erythematosus (SLE), who developed clinical and echocardiographic signs of hypertrophic cardiomyopathy. Neither a family history of cardiomyopathy or sudden death nor a personal history of hypertension or valvular lesions were present. The association of SLE with hypertrophic cardiomyopathy has been previously described in only 2 patients.