Juvenile active ossifying fibroma is a rare lesion seldom seen by neurosurgeons. It originates in the paranasal sinuses during childhood, grows slowly, and encroaches on adjacent orbital and cranial compartments. In the past 3 years, four patients with this lesion were seen (three men and one woman; mean age 28 years). The clinical presentations were different with each patient: sinusitis, meningitis, periorbital pain, and a unique case of a juvenile active ossifying fibroma presenting with high-grade internal carotid artery stenosis and ischemic symptoms. Three patients were treated by transfacial approaches: two with a transfrontal-nasal approach and one with a transfrontal-nasoorbital approach. Two open resections resulted in gross-total excision and no recurrence as of the 2-year follow-up review. In the third patient, the tumor-encased carotid artery was preserved at the expense of a complete resection; that patient underwent superficial temporal artery-middle cerebral artery bypass and remains without ischemic symptoms or tumor recurrence at 2 years. The fourth patient underwent three subtotal endoscopic resections and is also without symptomatic recurrence at 2 years. Three points must be made concerning these lesions. First, the clinical and radiographic characteristics of juvenile active ossifying fibroma may not be easily recognized by neurosurgeons, which could lead to misdiagnosis and mismanagement of these lesions. Second, this tumor can encase the carotid artery and cause severe stenosis or occlusion. Third, complete resection of the tumor is required to effect a cure, and transfacial approaches, which give wide exposure of the sinuses, appear to yield better, more radical resections than endoscopic procedures.