Carcinomas of the external auditory canal are a rare condition, accounting for approximately 0.2% of all tumors in the head and neck region. Their rarity explains the lack of reliable epidemiological data and the absence of consensus treatment recommendations. Diagnosis is based on imaging and histology, supplemented by the modified Pittsburgh classification, currently the most widely used method for assessing tumor spread. Management remains complex due to the lack of solid prognostic criteria and the diversity of possible therapeutic approaches. Surgery remains the standard option, although it is often invasive and associated with significant postoperative morbidity. Adjuvant radiotherapy is frequently offered, while the role of chemotherapy and exclusive chemoradiotherapy remains debated, particularly in locally advanced forms. Despite therapeutic advances, the prognosis for locally advanced forms remains poor. The main objective of our study is to present, through this clinical case, the diagnostic and therapeutic characteristics of CEAC, as well as to identify the main prognostic factors associated with this rare disease.