ABSTRACT: BACKGROUND: Gradenigo's syndrome is a rare disease, which is characterized by the triad of the following conditions: suppurative otitis media, pain in the distribution of the first and the second division of trigeminal nerve, and abducens nerve palsy. The full triad may often not be present, but can develop if the condition is not treated correctly. CASE PRESENTATION: We report a case of a 3-year-old girl, who presented with fever and left-sided acute otitis media. She developed acute mastoiditis, which was initially treated by intravenous antibiotics, ventilation tube insertion and cortical mastoidectomy. After 6 days the clinical picture was complicated by development of left-sided abducens palsy. MRI-scanning showed osteomyelitis within the petro-mastoid complex, and a hyper intense signal of the adjacent meninges. Microbiological investigations showed Staphylococcus aureus and Fusobacterium necrophorum. She was treated successfully with intravenous broad-spectrum antibiotic therapy with anaerobic coverage. After 8 weeks of follow-up there was no sign of recurrent infection or abducens palsy. CONCLUSION: Gradenigo's syndrome is a rare, but life-threatening complication to middle ear infection. It is most commonly caused by aerobic microorganisms, but anaerobic microorganisms may also be found why anaerobic coverage should be considered when determining the antibiotic treatment.