Odontogenic keratocyst (OKC) is an odontogenic developmental cyst that presents distinct clinical behavior. This lesion has been described as dental cysts with keratinization since the 1930s, however the term "OKC" was established in 1956. This study aims to determine the frequency and features of OKC in children aged 0 to 14 years in an oral pathology service in Brazil. A retrospective study was performed to review cases of OKC in children diagnosed between 1986 and 2017. Clinical data were evaluated from medical records (gender, race, age, anatomical location, treatment, radiographic findings and follow-up). Ninety-seven cases of OKC were diagnosed in a 31-year-period in all age groups and 10 were found in children (10.3%). Age ranged from 2 to 14 years (mean age=10.5±3.5), with 8 males and 2 females. The most frequent location was the anterior region of the mandible (n=4). Patients were predominantly asymptomatic. Moreover, in two children, clinical findings of Gorlin-Goltz Syndrome were observed. The most commonly used treatment was enucleation followed by curettage. In all cases of Gorlin-Goltz Syndrome were observed recurrences and occurrence of new keratocysts. Although uncommon in pediatric patients, OKC should be considered a differential diagnosis in cases of osteolytic lesions in gnathic bones. Thus, the periodic assessment of children by dentists and pediatricians is essential to get a correct diagnosis and early treatment to avoid greater mutilation of these patients.