The clinical picture of desmoids is unpredictable, which is a feature of different tumor specific associations. Anatomic location, age, sex, association with FAP as well as other factors determine biological behavior of the tumor. Negative prognosis is linked with the intraabdominal area and as many as 80% of desmoids are associated with FAP. Currently, biological targeting therapy is used in the treatment of many cancer diseases. It is only the question of time when and by which of these therapies we will be able to treat the patients with aggressive fibromatosis as well. A disadvantage is heterogenity and rare occurrence of desmoids. The efficacy of tailoring treatment still depends on knowledge and study of particular disease biological markers, which is currently the most important issue. .