The incidence of candidiasis has risen in neonatal intensive care units as advances in medical therapy have allowed for increased survival of extremely preterm neonates. The mortality of candidiasis has been reported to be 20% by several multi-centre studies. Definitive guidance for prophylaxis and treatment is hindered by lack of large, multi-centre, randomised controlled trials. Systemic prophylaxis is currently not recommended for any neonatal population, and amphotericin B deoxycholate continues to be used as first-line therapy for the treatment of invasive disease.