Invasive Candia infections have become the third most common cause of late-onset infection among very low birth weight infants in most neonatal intensive care units. Significant risk factors include birth weight less than 1000 g, exposure to more than two antibiotics, third generation cephalosporin exposure, parenteral nutrition including lipid emulsion, central venous catheter, and abdominal surgery. The majority of neonatal Candida infections are caused by C. albicans and C. parapsilosis, although other nonalbicans species are being reported more frequently. Standard therapy has been amphotericin B; however, successful use of fluconazole as a single agent has also been reported and a small comparison trial demonstrated similar efficacy. The addition of new antifungal agents, including voriconazole and the echinocandins may further improve our ability to effectively treat these infections and possibly reduce the development of complications. Antifungal chemoprophylaxis has been studied in single-center and cohort studies, primarily using fluconazole. Although it is clear that fluconazole prophylaxis decreases the risk of fungal colonization and infection, identification of potential harm, particularly the development of or selection for resistant strains, requires further investigation with multicenter trials before widespread use is recommended outside of the clinical trial setting.