Abstract: Aggressive fibromatosis (AF) is a clinically rare borderline tumor originating in soft tissue. Although AF does not have the ability of distant metastasis, it is locally aggressive and has a high recurrence rate after surgical resection, which makes clinical treatment difficult. In recent years, with the continuously deepening research on AF, great changes have taken place in its treatment measures and management models. Due to the unpredictable natural history of AF, wait-and-see is currently the first-line treatment recommended by the guidelines. In addition, the current multidisciplinary comprehensive diagnosis and treatment mode which mainly focuses on surgical radical resection combined with local and systemic treatment can control the tumor to the greatest extent, delay the progress of the disease and even achieve radical treatment while preserving the function of the tissues and organs. This article reviews the clinical features and the research progress in diagnosis and treatment of AF.