OBJECTIVES: We describe 10 new cases of otogenic (n = 8) and nonotogenic (n = 2) skull base osteomyelitis (SBO) in previously healthy children and review the literature on SBO in the pediatric population. METHODS: We retrospectively analyzed the medical records of 10 children (age range 0.9-12.8 years) discharged with a diagnosis of SBO between 2015 and 2020 in 2 children's hospitals in central Israel. RESULTS: Five patients presented with fever and 5 with otological signs and symptoms. All 10 children underwent a comprehensive clinical evaluation, imaging studies (computerized tomography or magnetic resonance imaging) and laboratory investigations. The physical examination revealed neurologic findings, including nuchal rigidity, papilledema, and apathy, in 4 patients. All 8 otogenic patients underwent surgical intervention and the 2 nonotogenic patients, who were diagnosed as having deep neck and throat infections, responded well to treatment consisting of antibiotics without surgery. CONCLUSIONS: Early diagnosis of pediatric SBO can be challenging because the symptoms are often nonspecific. The final diagnosis relies mainly on imaging, preferably magnetic resonance imaging. Surgical intervention is usually mandatory in the otogenic patients, whereas the nonotogenic patients respond well to medical management alone.