OBJECTIVE To present the first documented case of an angiolipoma of the maxillary sinus, our treatment of the patient, and the 1-year follow-up results. At the conclusion of the report, the reader should be able to discuss the incidence, treatment options, and long-term prognosis of angiolipomas of the paranasal sinuses. METHODS A case study reporting on a single patient with the diagnosis of maxillary sinus angiolipoma. METHODS Review of diagnostic studies, the operative technique, and the patient's chart for the 1-year period after treatment. RESULTS The patient remains free of disease 1 year after definitive surgical treatment. CONCLUSIONS Angiolipomas are exceedingly rare in this location. The accepted treatment for this disease in other regions of the body consists of wide local excision with close follow-up. The extent of the disease found in this patient required a medial maxillectomy for en bloc resection. We would advocate such an approach for other physicians who are faced with a patient with this rare diagnosis.