Calcifying aponeurotic fi broma is a benign tumor with a predilection for distal parts of the extremities; it is very rare in the head and neck region. It commonly aff ects young patients-hence the term juvenile in the name. It is fi broblastic in origin and considered a cartilage analogue of fi bromatosis, but its exact etiology remains unknown. Clinically, this tumor needs to be diff erentiated from fi bromatosis, nodular fasciitis, chondroma, schwannoma, and rheumatoid nodule. A 24-year-old woman presented with swelling in the forehead for the previous 6 months. Wide surgical excision of the lesion was performed, and histopathologic examination revealed a calcifying aponeurotic fi broma. We reviewed the literature on this rare clinical entity and present our hypothesis on its etiology.