A rare case of angiosarcoma of the maxillary sinus is reported. Review of the literature concerning angiosarcoma of the head and neck revealed 14 previously published cases involving the nose, nasal sinuses, and nasopharynx. Angiosarcoma of the skin and soft tissues of the head and neck differs in behavior from that affecting the nose, nasal sinuses, and nasopharynx. The case reported presents the protracted clinical course of this malignant tumor.