Fibrous dysplasia of bones comprising the paranasal sinuses is well recognized. Depending on the anatomic areas of the skull involved by this neoplasm, a combined approach for surgical removal may be necessitated. A case of a patient with fibrous dysplasia of the ethmoid sinus, possibly the first reported case of such change in the ethmoid bone itself, is presented. A two-stage, combined otolaryngologicneurosurgical craniofacial resection was the method of treatment.