OBJECTIVE: To describe a case of rapidly growing, ossifying fibroma requiring consecutive surgical procedures. CASE REPORT: J. A. O., a 6-year-old male child, visited the otorhinolaryngology department of HU / Sergipe in January 2012 for an assessment of a frontal headache and left facial pain associated with nasal obstruction and facial asymmetry. CT imaging of the sinuses revealed a circumscribed, hyperdense lesion in the left ethmoidal maxillary region. He underwent excision of the lesion in May 2012 via a combined approach (endoscopic and expanded Caldwell-Luc), but without removal of the tumor in the posterior ethmoid region because of the risk of injury to the optic nerve. The pathology confirmed a diagnosis of ossifying fibroma. After 4 months, the patient returned with facial deformity, and a new CT scan showed growth of the ossifying fibroma, with bulging of the medial orbital wall. The patient underwent a new procedure with the same combined method 7 months after the first surgery, leaving a remnant lesion near the optic nerve. A decision was taken to not perform postoperative CT to avoid subjecting the patient to more radiation within a short period of time. Four months after the second procedure, the patient returned to the clinic with facial pain, epiphora of the left eye, and facial asymmetry with CT imaging showing an extensive, hyperdense area in the left hemifacial region and ocular proptosis, but without a loss of visual acuity. The patient remains in a preoperative status, awaiting a new surgical procedure. CONCLUSION: Ossifying fibroma, although benign, can have an aggressive nature, requiring close monitoring and often a series of surgical procedures within a short time.