Abstract: Aggressive fibromatosis (AF), also known as desmoid-type fibromatosis, desmoid tumor (DT), or muscle aponeurotic fibromatosis, among others, is a tumor that can be either benign or malignant. AF without a capsule usually locally infiltrates surrounding tissues, may be multifocal, and often recurs after surgery; however, it is not prone to metastasis. The current treatments for AF mainly include active surveillance, surgical treatment, radiotherapy, chemotherapy, targeted therapy, and endocrine and non-steroidal drug therapy. In the past, targeted therapy had limited efficacy for AF, and its research progressed slowly. Therefore, it was usually used as salvage treatment after other conventional treatments failed. However, in recent years, with the rapid development of basic research on targeted therapy, some new targeted drugs have been increasingly used as clinical treatment for AF and have achieved good clinical curative effects. Therefore, predicting the curative effects of these targeted drugs on AF and understanding their therapeutic mechanisms are currently hotspots of research on targeted therapy for AF. This study reviews the current progress of research on targeted therapy for AF.