Cardiac involvement in disseminated fungal infection is a rare condition among neonates with an increasing occurrence rate over the past years. Candida species represent the most common pathogen (66%) of fungal endocarditis for this class of patients. The disease is usually found in infants with an underlying immunodeficiency such as premature infants who receive corticoid therapy and neonate patients with preexisting cardiac pathologies. Fungal endocarditis has a high mortality rate due to the diagnostic difficulties represented by non-specific clinical manifestations, a low incidence of positive blood cultures, limited use of antifungal drugs and restrictions of surgical management. The purpose of this article is to evaluate the acquirements regarding this pathology that are stated in literature and to present the therapeutic options available at this point.