Carcinoma of the external and middle ear is a very rare disease. Despite the various treatment modalities available, its prognosis is still poor. We aimed to analyze the treatment modalities, outcomes, and validity of surgical approaches, especially in advanced tumors in the ear. Twenty-five patients with carcinoma of the external and middle ear were retrospectively analyzed. The modified Pittsburgh staging system was used for staging of the patients. Overall and disease-free survival was estimated using of Kaplan-Meier method. In our cohort of 25 patients, the majority (76%) had tumor located in external auditory meatus. The most common histologic subtype was squamous cell carcinoma (80%). More than half of patients (56%) had tumor stage IV. In the stage IV group, five patients underwent subtotal temporal bone resection and ipsilateral neck dissection. Seven patients underwent definitive radiotherapy. The remaining two patients underwent palliative chemotherapy. The 2-year overall and disease-free survival for patients with tumor stage IV was 67.7% and 57.8%, respectively. In patients with tumor stage IV, the 2-year overall survival for patients who underwent surgery was 80.0% versus 53.6% for those who underwent radiotherapy (P = 0.16). The 2-year disease-free survival for patients who underwent surgery was 80.0% versus 28.6% for those who underwent radiotherapy (P = 0.15). In the present study, the outcome of patients who received surgical treatment tended to be better than that of patients who received radiation therapy. Our results suggest that en bloc resection could be the first choice even in the advanced disease stage.