Desmoid tumors are non-metastatic mesenchymal tumors with an aggressive local growth. Depending on the anatomic location, morbidity varies. The successful treatment of desmoid tumors requires an interdisciplinary approach. There is a need for prospective randomized studies with larger numbers of patients to allow the development of standardized and evidence based treatment algorithms. We report of a child with a desmoid tumor of the left shoulder which was treated in our department by surgical excision.