Candida species can cause clinical manifestations in various organs of the cardiovascular system, i.e., the pericardium, myocardium, and endocardium, with endocarditis being the best-known clinical entity. Endocarditis is seen primarily in intravenous drug users and in individuals with damaged native valves, especially in congenital heart disease or rheumatic valvular diseases, and in prosthetic heart valves. The authors present a case of Pichia ohmeri endocarditis in an intravenous drug user, with an unusual presentation form. This is a case of a 42-year-old man, an intravenous heroin user, who was admitted to our Vascular Surgery Department because of fever and acute serious ischemia of the left inferior limb. He presented with fever (39 degrees C), a pale and cold left limb, absence of the left popliteal pulse, and a pansystolic murmur at the cardiac apex. The transthoracic echocardiogram showed a large vegetation on the anterior leaflet of the mitral valve and severe mitral regurgitation with good left ventricular systolic function. Empirical antibiotic therapy was started. Six days after admission, embolectomy was performed with partial clinical recovery. Three blood cultures and the embolus showed a teleomorphic form of Candida guilliermondii - Pichia ohmeri. Therapy with intravenous liposomal amphotericin B, fluocitosin, imipenem, and aztreonam was started. Two weeks later, his clinical condition deteriorated with acute heart failure refractory to medical therapy, mandating mechanical ventilation and high-dose vasopressor and inotropic amine support. He underwent urgent mitral valve replacement with a biologic prosthetic valve. Rapid stabilization of the cardiac status occurred, but ischemic limb lesions required further vascular interventions.