Comparative review of the limited literature shows that most previously reported angiofibromas outside the nasopharynx arise in the extremities or pelvic soft tissue, often in middle-aged adults, and share similar histologic and molecular features, particularly the AHRR-NCOA2 gene fusion, thereby excluding other spindle cell neoplasms [1,2]. This evidence concerns the gene NCOA2 and Angiofibromas.