We present our experience of two cases of rare connective tissue disease occurring within the midface. First case represented a very large painless slowly growing neck mass extending from mid neck to the clavicle displacing the trachea to the other side. Second case represented a painful mass in the right posterior mandibular region that had overgrows teeth. FNA and incisional biopsy did not confirm the diagnosis. A complete excision of the lesion with invasion of adjacent structure confirms the diagnosis of Aggressive Fibromatosis. Different modalities of the treatment will be discussed