Paranasal sinus cancers are rare diseases, accounting for about 5% of all head and neck malignancies. The variety of histological types and the overlapping pathological features with other entities constitute difficulties in pathologic interpretation, often requiring a skilled interpretation or a second opinion. Treatment of locally advanced disease relies on surgery and radiation therapy for operable disease, with a possible role for systemic treatment in selected histologies within a multimodal approach; unresectable paranasal sinus cancers are generally treated with a combination of radiotherapy and chemotherapy. The employment of high conformal radiation techniques, such as Intensity Modulated Radiation Therapy orcharged particle therapy, proton or carbon ion therapy may improve outcome and reduce late effects. Surgical treatment has evolved due to the progressive application of transnasal endoscopic techniques for naso-ethmoidal malignancies and due to innovative reconstructive techniques after resection of cancers of the maxillary sinus. Because of the rarity and complexity of this disease, multicenter trials represent an urgent need to improve prognosis and to reduce treatment-related effects.