The LEOPARD syndrome is a dysmorphogenetic and multisystem cardio-cutaneous syndrome. A 32-year-old female is presented with multiple lentigines, hypertrophic obstructive cardiomyopathia (HOCM), tight bundle branch block, mitral valve stenosis and insufficiency, scoliosis, ocular hypertelorism, gynocologic pathologies and iron deficiency anemia. Hyperthrophic cardiomyopathy was treated by septal ablation two years ago. Hyperthrophic cardiomyopathy was also diagnosed in her sister without lentigines and there was no family history of LEOPARD syndrome in the family. Patients with lentiginous lesions must be considered for systemic disease and should be investigated for cardiac disorders.