Malignant external otitis in the pediatric population is primarily a disease of children with chronic illness or immunosuppression. The presence of severe, unrelenting otalgia, otorrhea with isolation of Pseudomonas aeruginosa, a markedly elevated erythrocyte sedimentation rate, and evidence of bone destruction on computed tomography scan should alert the clinician to the diagnosis. Unlike adults, children have a higher incidence of seventh nerve paralysis earlier in the course of the infection. They also manifest more frequent involvement of the middle ear with tympanic membrane destruction. The short interval between the onset of symptoms and facial nerve dysfunction highlights the necessity of prompt diagnosis and institution of anti-Pseudomonas therapy. Our review suggests that this destructive bacterial infection is an emerging clinical entity in children; 73% of the cases have been reported since 1980. Pediatricians should therefore be familiar with the clinical presentation of this treatable infection. Substantial morbidity could be alleviated by prompt diagnosis and early antibiotic treatment.