To identify predisposing factors, species distribution, antifungal susceptibility, and outcome. Methods: This study is a retrospective chart review that was conducted at a children's hospital at King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia. One hundred twenty-nine children with invasive candidiasis who were admitted between January 2010 and January 2015. Results: The statistical analysis results have revealed a group of risk factors; prematurity in 37 (28.7%) of patients, low birth weight in 42 (32.6%), central venous catheter in 59 (45.7%), malignancy in 21 (16.3%), immunotherapy in 20 (15.5%), and ventilator support in 60 (46.5%). More than 2-fold mortality rate in patients who had heart vegetation (odds ratio [OR]: 2.9) and patients who had Candida isolated from their blood were more than twice as likely to die as patients with Candida isolated from other sites (OR: 2.2). A total of 48.3% of patients on ventilator died versus 26.1% who were not on ventilator (p=0.009); and 43.8% of patients in the ICU died versus only 24.5% of patients who were not in the ICU (p=0.03). Candida parapsilosis exhibited the highest mortality rate (56.2%). Conclusion: Candida albicans is the most common isolate among all Candida species. Gender, low birth weight, prolonged ICU stay, presence of vegetation, positive blood culture, and mechanical ventilation as a strong predictive risk factors for death in children with invasive candidiasis, a finding that could be applied as prophylactic indicator in critically ill children especially neonates.