Desmoid tumours are rare monoclonal fibroblastic proliferations arising in the soft tissue and accounting for less than 0.03% of all neoplasms. These benign neoplasms are locally invasive and tend to recur, but do not metastasise. Desmoid tumours generally occur between the ages of 15 and 60 years. [1,2] Two different types have been described: a sporadic type, and a familial type in which the tumours are usually intraabdominal. [2,3] The chest wall is the most common site for extraabdominal desmoid tumours, with true intrathoracic tumours being extremely rare. Patients with intrathoracic desmoid tumours are usually asymptomatic and generally present when the lesion is large enough to cause compression of vital structures or erosion into adjacent bone or joints. These tumours resemble sarcomas both clinically and histologically. Although not specific, beta-catenin and mutations thereof are highly sensitive for sporadic desmoid tumours. [3] Firstline therapy is complete surgical resection if this will not result in unacceptable morbidity.