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Desmoid tumours are uncommon mesenchymal neoplasms with a fibrotic band-like consistency. They are also called aggressive fibromatosis tumours which are locally aggressive with no potential for distant metastases. The important causes of morbidity and mortality are local recurrence and adjacent organ involvement. Desmoids can be of three types, namely intra-abdominal, in the abdominal wall, or extra-abdominal. Treatment options include observation, surgical resection, radiotherapy, conventional chemotherapy, hormonal agents and newer molecular-targeted agents. A multidisciplinary approach tailored to the individual patient is usually needed, depending on the location, local effects and the clinical course of the disease.

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