Advances in medical therapy have increased premature infant survival. A rise in Candida infections in neonatal intensive care units (NICUs) has followed. Once considered a contaminant, Candida is now recognized as a major cause of mortality and morbidity within these units. We will examine what is known about the epidemiology, risk factors and end-organ involvement of Candida infections with a focus on invasive disease. In addition, diagnostic alternatives to traditional blood culture, experience with antifungal agents for prophylaxis and therapeutic options, including newer antifungal agents, will be presented.