Malignant otitis externa (MOE) is a rare, rapidly spreading and invasive infection of external ear that has the propensity to spread to surrounding structures. Skull base osteomyelitis (SBO) is a rare fatal infection of the base of skull with otologic or sinogenic cause. MOE and SBO are usually infection of the immunocompromised elderly individuals of which diabetics are the most commonly affected. The protocol-based treatment strategies are lacking even though skull base osteomyelitis has been prevalent for a long time. In view of this, our review was performed to identify the various treatment strategies for malignant otitis externa and skull base osteomyelitis. This systematic review has been conducted as per PRISMA (preferred reporting items for systematic review and meta-analysis) [1]. Pubmed and Scopus databases were used to identify the eligible articles and the period of search was from January 1st 2015 to June 30th 2025. The phrases or MeSH terms for the search included malignant OR necrotizing AND otitis externa AND skull base osteomyelitis AND/OR atypical OR central skull base osteomyelitis AND/OR treatment. The articles which included both typical and central skull base osteomyelitis were also included. Articles with at least one or more defined treatment modalities like antibiotic treatment, antifungal treatment, surgical intervention, hyperbaric oxygen therapy (HBOT) were included. The quality of the included studies was assessed by MINORS (Methodological Index for Non-randomized studies) criteria. Out of 16 included studies, 11 were prospective, 4 were retrospective and 1 was combined retrospective and prospective study. Total study sample is 779 patients. Total patients with MOE were 383 (49.2%), typical SBO were 353 (45.3%) and atypical or central SBO were 43 (5.5%). The mainstay of treatment in our review was antibiotics (100%) and dual antibiotics were started in 13 out of 16 studies. Antifungal treatment was started in cases who are refractory to antibiotic therapy or when culture revealed fungal aetiology. In our review, 103 (13.2%) patients underwent surgery. The details regarding the surgical management were lacking and it ranged from simple deep biopsies or abscess drainage to debridement and in some cases radical mastoidectomy or sub-total petrosectomy. Our review has found that SBO is a medical disease generally treated with parenteral antibiotics. Surgical treatment is limited to adequate debridement or deep biopsies or in cases with intracranial or cranial nerve complications.