Fibromatosis is a broad group of benign fibroblastic proliferation that shows locally aggressive growth but never metastasize. Common anatomic sites include abdominal wall, extremity, and mesentery. Little is reported about clinical features and outcome of fibromatosis of the head and neck. The treatment of choice is wide excision, which is often difficult. Postoperative recurrence rates are high. We recently confirmed a unique case of fibromatosis occurred on the hypopharynx of 44-year-old male patient and report this interesting case with review of literature.