Inflammation of a part or whole of the temporal bone and surrounding soft tissue is termed as malignant otitis externa, which typically spreads to skull base to involve cranial nerves VII. Rarely can it also effect one or more of cranial nerves IX, X, XI, and XII. We present a case of malignant otitis externa which presented with symptomatic palsy of IX and XII nerves sparing the VII cranial nerve. The patient though later on had internal jugular vein thrombosis, which we presume is due to the involvement of the parapharyngeal space that prompted us to reconsider the diagnosis, and later on, to aggravate the therapy. With proper blood sugar control and appropriate long term antibiotics, not only that the patient is disease free at one year follow up, but the cranial nerve deficits also recovered. Apart from sharing the clinical and management details of this patient, we have reviewed the relevant literature in the discussion, which has shed some light onto some of the interesting facts about this condition and its prognosis.