Fungal endocarditis in premature infants has rarely been reported; only 16 cases have been described. We present a fatal case of endocarditis due to Candida albicans in a patient requiring neonatal intensive care and parenteral nutrition via a central venous catheter; there were no preexisting pathological abnormalities. The patient had sepsis with multisystem organ failure, resulting in death. A postmortem examination was performed, and the most important findings are presented. The literature on fungal endocarditis in premature infants is reviewed as well.