Malignant otitis externa (MOE) also known as necrotizing otitis externa, is a rare but potentially fatal infection that impacts the external auditory canal and nearby structures, associated with skull base soft tissue infection and osteomyelitis. This condition is most commonly seen in diabetic or immunocompromised individuals, especially those aged 65 and above. Pseudomonas aeruginosa is the most frequently identified pathogen, although other bacteria and fungi have been found as well. Symptoms of NOE include persistent ear pain, chronic ear discharge, sensation of fullness in the ear, hearing loss, headaches, and involvement of the cranial nerves. The diagnosis is usually made through a combination of patient history, physical examination, lab tests, and imaging studies. An otoscopic examination may show inflammation in the external auditory canal, along with polyps or granulation tissue. Inflammatory markers such as white blood cell count, C-reactive protein (CRP), or erythrocyte sedimentation rate (ESR) may be elevated. Imaging studies like Computed tomography (CT) and Magnetic resonance imaging (MRI) scans can help identify involvement of bone and soft tissue, respectively. A positive result on a methylene diphosphonate (MDP)-technetium-99 m (Tc99m) bone scan and Gallium-67 (Ga67) scan can further support the diagnosis of NOE. Complications can include osteomyelitis, meningitis, brain abscess, and cranial nerve palsy. The primary treatment is long-term systemic antibiotics targeting Pseudomonas, but in aggressive or advanced cases, or those not responding to antibiotics, surgical debridement may be necessary.