Imatinib, a tyrosine kinase inhibitor, is a major therapeutic option for the management of unresectable aggressive fibromatosis (AF) or desmoid tumors [
1]. These tumors are non-malignant and aggressive; they can occur anywhere in the body. Extra-abdominal forms are usually confined to the musculature and the overlying aponeurosis or fascia but the neoplasm may infiltrate the surrounding tissue up to 2 to 3cm outside the palpable tumor [
2]. Management of these tumors is not standardized but relies on the combination of surgery, radiotherapy and/or systemic therapy. Local control is the main goal of treatment and there has been a change in the management of these tumors from aggressive surgical resection to function preservation [
3]. The surgical resection rate regarding primary treatment modality for desmoid tumors when functionally and cosmetically acceptable with reported local control is 75 to 80% [
3]. For surgery alone, local recurrence rates varied from 24 to 77% which justified the use of other therapeutic options. Systemic therapy has been reported regarding cytotoxic agents [
4] but with documented cardiotoxicity and myelosuppression. Regarding the relative toxicities of cytotoxic agents, hormonal therapy and tyrosine kinase inhibitors are increasingly reported as therapeutic options [
5]. Imatinib mesylate (Gleevec
®) is a specific tyrosine kinase inhibitor highly used for targeting c-KIT, breakpoint cluster region-abelson gene (
BCR-ABL), platelet-derived growth factor receptors (PDGFRs) and macrophage colony-stimulating factor receptor. Longtime disease stabilization with imatinib mesylate has been reported in different series of patients with relapsing desmoid tumors, with 1-year progression-free survival rates close to 60 to 70% [
6‐
8]. Unfortunately, for most patients of low or very low average income countries, surgery often is the first treatment option. This is related to the unavailability of chemotherapy or targeted therapy, and a lack of financial resources or lack of surgeons’ knowledge of other therapeutic options. Although AF tumors are benign, the best management of AF is a multidisciplinary approach to plan local control with acceptable morbidity.