Skull base osteomyelitis is a rare but dangerous consequence of untreated malignant otitis externa. Pseudomonas aeruginosa is responsible for most cases with typical presentation. Here, we discuss a diabetic 54‐year‐old female presented with malignant otitis externa and bilateral facial paresis followed by cervical spondylitis and C1‐C2 instability. Skull base osteomyelitis confirmed by clinical presentation, imaging, and laboratory data. Fortunately, she responded well to antibacterial and antifungal therapy. Due to limited data, there is no confirmed standard of treatment for cervical instability secondary to SBO. It seems antibiotic therapy is the mainstay of treatment. In case of poor response to antibiotic therapy, surgical intervention is inevitable. This article introduces the first case of SBO‐related AAI successfully managed with conservative treatment.