Invasive fungal disease (IFD) is an important infection with high rates of morbidity and mortality in hospitalized patients. Data on incidence, risk factors, and mortality of IFD in the pediatric population, mainly in South America, are scarce. The aim of this paper was to review the literature about the most important IFD in pediatrics in South America. We searched three electronic databases (Medline, Lilacs, and Cochrane databases) for studies published between 2012 and 2015; case reports and editorial were excluded. Twenty-two articles were found on Candida spp. infections; eight on Paracoccidiodes spp.; two on Cryptococcus spp.; and one on Aspergillus spp. Candida albicans was the main agent, followed by Candida parapsilosis in pediatric population. Paracoccidioides spp. had a prevalence ranging from 2.3 to 35.3 % with ages between 11 and 29 years, malnutrition, and hepatic involvement related to the worst prognosis. Cryptococcus spp. showed a prevalence of 2.6 % in under 16 years old, with cryptococcal meningitis most observed, mainly by Cryptococcus neoformans (94.1 %). Aspergillosis and other mold infections, as zygomycosis and fusariosis occurring mostly in immunocompromised children, related with relevant morbidity and mortality in this population