(1) In recent years, a global epidemiological shift in candidemia has been observed, marked by the emergence of resistant non-albicans Candida species. , in particular, has become a significant global concern, causing infections in both pediatric and adult populations within healthcare settings. Despite its widespread impact, there is a limited understanding of the clinical course and transmission dynamics of neonatal systemic Candida auris infections, hindering effective prevention and management. This study focused on the epidemiologic data, the clinical presentation, risk factors, and outcome of infection in neonatal population. (2) : A systematic review of the literature using PubMed and Scopus databases until December 2023 was conducted. (3) : A total of 24 relevant studies were identified, encompassing 476 documented cases of infection in neonates. Prematurity emerged as a primary risk factor, alongside total parenteral nutrition, central line insertion, mechanical ventilation, and prior broad-spectrum antibiotic use. The mortality rate reached approximately 42%, with therapeutic details sparingly reported in 12% of cases. Treatment strategies varied, with amphotericin B predominantly used as monotherapy, while combination antifungal agents were used in 44% of cases. Notably, 97.4% of cases exhibited fluconazole resistance, and 67.1% showed resistance to amphotericin B. Limited data were available on resistance to other antifungal agents. (4) : Despite the rarity of neonatal infections, their global occurrence necessitates comprehensive preparedness in patient care. A deeper understanding of pathogenesis is crucial for developing effective strategies to control and prevent neonatal infections caused by this pathogen.