Angiofibroma is an uncommon, highly vascular, locally invasive, non-encapsulated tumor with a sharp, if not almost exclusive, predilection for origin in the posterior nares or nasopharynx of adolescent males. Angiofibroma developed in soft palate is a rare case, so there is no description in several textbooks, but only one case was reported in the medical journals up to date. Recently we experienced a case of angiofibroma developed in the right soft palate combined with a huge arteriovenous malformation in opposite temporal lobe area, so we report its clinical manifestation and characteristics with a review of literature.