Background: Invasive fungal infections are common opportunistic diseases in patients with AIDS, other conditions related to immunodeficiency and healthy infants. Most publications on this subject are related to industrialized countries, and in adult population, with limited data in Latin America (except for Brazil, Colombia, and Argentina), and especially in pediatric population. These patients present a variety of clinical manifestations representing a diagnostic and therapeutic challenge for the health system. Objective: The objective of the study is to describe the epidemiological and laboratory characteristics of children with invasive fungal infections in Guatemala. Methods: A review of the microbiology service database was carried out at Roosevelt Hospital in Guatemala. Positive cultures were taken from children under 15 years of age, in a period of seven years, from 2007 to 2014, with its corresponding medical history. Results: Finally, 23 isolates were documented but only 15 patients were included in the study with complete information; 10