Abstract： Objective To study the clinical features and treatment of aggressive fibromatosis ( AF). Methods One hundred and nine the clinical characters, treatment and efficacy of each patient were reviewed. Results One hundred and nine pathologically diagnosed AF patients presented in Peking Union Medical College Hospital ( PUMCH) was analyzed. 70 females and 39 males were included with an average age of 34.0 and 36.9 yrs respec-tively. 87.2% of patients first presented as mass, 33.7% first presented as mass accompanying pain. 32. 1% of mass located in the abdomen wall among which 94. 3% were female, 51.5% of these females had a history of sur-gery of the abdomen. Diameter of all the masses was(7. 84 ±5.62)cm, diameter of deep mass was statistically lar-ger than that of superior ones, which were(11. 1 ±5.9)cm and(6. 8 ±5. 1 )cm respectively,P <0. 01. Twelve pa-tients got radiotherapy and 8 of them did it for the purpose of preventing disease recurrence. 7 out of them recurrent within an average interval of about 2. 7 years. A patient whose tumor was located in the anterior mediastinum was treated with chemotherapy and had a partial response. 4 patients died of AF. Conclusion Operation is still most widely used therapeutic option. Radiotherapy is also effective in certain percentage of patients and medicine such as chemotherapy or NSAlDs that have not been receipted.