Introduction: Fibromatosis is a very rare desmoid tumour. It is characterised by local invasion and infiltration of close structures. The treatment is mainly surgical. Material and methods: We report the case of 1.5-year-old boy admitted to the Paediatric Hospital, with a diagnosis of mediastinal desmoid fibromatosis. He was treated surgically (thoracoscopy and thoracotomy), chemotherapy was used, and he was hospitalised in the Intensive Care Unit more than once. Despite intensive treatment, the patient perished. Results and conclusions: Fibromatosis is classified as a desmoid tumour. Rare tumour localisation and invasive tumour characteristics causes considerable difficulty in the diagnosis, despite the use of many diagnostic methods.
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