Ethmoid sinus cancer typically demonstrates a propensity for late diagnosis and poor prognosis. This study confirms that craniofacial resection plus combined associated therapy is the optimal approach for the effective management of extensive ethmoid sinus tumors and is associated with an acceptable morbidity rate. More aggressive disease management featuring prophylactic concurrent chemoradiotherapy including neck or elective neck dissection plus chemotherapy should be considered for T3-T4 patients as opposed to T1-T2 patients.