AbstractAngiomatosis is a rare benign lesion of the head and neck that can be mistaken for either a vascular malformation or malignant disease as a result of its infiltrative nature. The recurrence rate of angiomatosis requiring surgery is reported to be >90%, and as such the otolaryngologist treating this condition should endeavor to remove all disease during the first operation while maintaining a high level of suspicion for recurrence during postoperative surveillance. This case represents the first report of angiomatosis involving the nose and/or paranasal sinuses, and extends the differential diagnosis of sinonasal tumors, of which the otolaryngologist must be aware. A description of the surgical care and postoperative surveillance recommendations is detailed in this first reported case of angiomatosis of the paranasal sinuses.