BACKGROUND: Fungal endocarditis (FE) remains an uncommon but life-threatening complication of invasive fungal infections. As data on neonatal FE are scant, we aimed to review all published experience regarding this serious infection.METHODS: Neonatal FE cases published in PubMed (1971-2013) as single cases, or case series were identified using the terms "fungal endocarditis, neonates and cardiac vegetation." Data on predefined criteria including demographics, predisposing factors, mycology, sites of cardiac involvement, therapy and outcome were collected and analyzed.RESULTS: The dataset comprised 71 neonates with FE. Median birth weight was 940 g [interquartile range (IQR): 609], median gestational age 27 weeks (IQR: 6) and median postnatal age at diagnosis 20 days (IQR: 20). Ninety-two percent of the patients were premature. Right atrium was the most common vegetation site (63%). Seventy-one percent of the cases reported were associated with previous central venous catheters. Candida albicans was the most predominant fungal species (59%). Amphotericin B monotherapy was used in 42.2% and fluconazole in 2.8%. Amphotericin B with flucytosine (25.3%) was the most frequent combined regimen. Surgical treatment was conducted in 28%. Overall mortality was 42.2%. Initiation with combined antifungal treatment was associated with lower mortality than monotherapy (24.2% vs. 51.7%, respectively, P = 0.036).CONCLUSIONS: Neonatal FE most frequently occurs in very premature infants and is associated with central venous catheters. C. albicans is the predominant fungus. Although outcome has been dismal, it may be improved with combined antifungal therapy.