Preterm and low birth weight (LBW) infants are vulnerable to invasive candidiasis, usually caused by Candida albicans and Candida parapsilosis. Neonatal candidiasis, acquired after 6 days of life, is the most common form of invasive candidiasis in neonates. Once the Candida species penetrate any organ system, it can lead to complications such as meningitis, endocarditis, pyelonephritis, septic arthritis and pneumonia. In recent years, the incidence of invasive candidiasis has decreased in neonatal intensive care units (NICU) due to improvements in neonatal care; however, in the affected neonates, mortality remains a major concern. This is primarily attributable to the limitations of currently used diagnostic tests and delay in treatment. In addition, there is a lack of data on pharmacokinetics and pharmacodynamics of various anti-fungal therapeutic regimens in neonates. This review provides an overview of invasive candidiasis in neonates, including modes of transmission, its risk factors, and management, with special focus on the recent updates in diagnosis and treatment.