This paper describes the clinical, radiographic and histologic findings of an aggressive infantile (desmoid-type) fibromatosis of the face in a seven-year-old black Jamaican male. This condition is rare in the head and neck region and its occurrence in the maxilla is exceptional. The differential diagnosis, management and long term follow-up of this case are also mentioned The need for a less aggressive surgical management in this child and long-term follow-up is stressed. Also, its occurrence in someone of African descent has not been reported previously. The absence of recurrence, eight years after surgery is significant. This paper discusses the differential diagnosis and treatment of aggressive infantile fibromatosis and suggests a classification of the condition.