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Introduction: Squamous cell carcinoma (SCC) of the temporal bone is a rare malignancy. Clear cell SCC is a rare variant of SCC of temporal bone in which no clear risk factor has been suggested as possible etiology. Otalgia, otorrhea, and hearing loss are the usual presenting symptoms of SCC of the temporal bone., Presentation of Case: This is a case is of a 62-year-old female who presented with a 6 months' history of experiencing intermittent left hearing disturbance, loss of balance, persistent left tinnitus, left otalgia radiating to the lateral neck and post auricular swelling. Histology showed clear cell variant, well differentiated SCC. The patient's tumor was found to be non-resectable due to the extensive invasion. The patient was referred for palliative therapy by medical and radiation oncology, however, the patient couldn't tolerate it. One month later she passed away., Discussion: SCC of the temporal bone is a challenging clinical entity. It is diagnosed mainly by clinical and radiological assessment, and deep biopsies are used to confirm the diagnosis. Temporal bone SCC is usually diagnosed late due to delayed presentation. This case of temporal bone SCC was aggressive in nature and presentation. The presentation was different from the known triad of symptoms of temporal bone SCC which is offensive otorrhea, pain, and bleeding., Conclusion: This case showed a very destructive and bizarre clinical presentation but more report of cases is needed to have a better characterization of the clinical presentation and prognosis of this variant of SCC of temporal bone., (Copyright © 2018 The Author(s). Published by Elsevier Ltd.. All rights reserved.)