PURPOSEFungal endocarditis in neonates poses a significant clinical challenge, with limited comprehensive data available. The aim of this study was describe a case report and further characterize demographic and clinical aspects of fungal endocarditis in neonates.METHODSWe present, a case report of a very low birth weight infant with fungal endocarditis. We also conducted a thorough analysis of 79 documented clinical cases, exploring demographics, clinical characteristics, treatment strategies, and outcomes.RESULTSThe temporal distribution revealed varied trends over the years. The United States had the highest case count, followed by India, Turkey, and Israel. Prematurity was predominant, with extremely low birth weight neonates being most affected. Congenital malformations were present in 10.2 % of cases. Surgical interventions, particularly for vegetative mass removal, were common. Candida albicans was the primary pathogen, followed by Candida parapsilosis. Catheter use was strongly associated with the majority of cases. The overall mortality rate was 38 %. Higher mortality was observed in neonates receiving monotherapy compared to those on combination therapy. Mortality was also significantly higher in neonates with prior bacteremia or fungemia and in those receiving antibiotic therapy without confirmed bacteremia. Conversely, postoperative mortality associated with surgery was 36.4 %, slightly lower than the overall mortality rate, suggesting that surgery may not represent an increased risk.CONCLUSIONOur study provides comprehensive insights into fungal endocarditis in neonates, highlighting the need for tailored management strategies to improve outcomes.