The aim of this study is to identify differences in distribution of Candida species, resistance to antifungals and clinical outcome, as well as the identification of potential risk factors associated with candidaemia in children. We conducted a retrospective analysis in children 18years with blood culture proven candidaemia identified between 2004 and 2012. Patients were divided into two groups (Group 1, <3months, n=51; Group 2, 3months, n=197) to identify any potential difference between the neonatal and early infantile periods in terms of risk factors and distribution of Candida species. A total of 248 distinct episodes of candidaemia were identified over the study period. The most frequently isolated Candida species were C. albicans (53.2%), followed by C. parapsilosis (26.2%), C. tropicalis (8.1%). Of the 248 episodes, 71 episodes (28.6%) resulted in death within 30days from the onset of candidaemia. In Group 1, failure of central venous catheter (CVC) removal was found to be associated with a 20.5-fold increase in mortality [95% CI (3.9, 106.5); P<0.001], compared to a 5.9-fold increased risk with hypoalbuminaemia [95% CI (1.03, 34.1); P=0.046]. For Group 2, the increased risk was 23-fold for failure of CVC removal [95% CI (7.48, 70.77); P<0.001], 7.4-fold for mechanical ventilation [95% CI (2.64, 21.08); P<0.001], 4.4-fold for hypoalbuminaemia [95% CI (1.56, 12.56); P=0.005], 3.1-fold for neutropaenia [95% CI (1.31, 7.69); P=0.010] and 2.2-fold for male gender [95% CI (1.02, 4.71); P=0.043]. Therapeutic choices should be guided by sound knowledge of local epidemiological trends in candidaemia. Removal of CVC significantly reduces mortality and is an essential step in the management of candidaemia.