Background: Desmoid tumor (DT) is a rare locally aggressive tumor of unpredictable evolution that develops in deep soft tissues with an infiltrative growth pattern and with a high tendency to local recurrence. Only 17% of extra-abdominal DT develops in the shoulder girdle. Aim: Describe the behavior and unpredictable evolution of DT as well as the different treatment options currently recommended, along with their advantages and disadvantages. Case Presentation: A 29-year-old female with a DT in the shoulder girdle of 6 years of evolution with progressive growth, pain, and limitation in the joint movements treated with chemotherapy presenting stable disease and switching to Tyrosine Kinase Inhibitors (TKI) with progressive disease for which a R0 resection was performed. Currently, under surveillance with a 3-year disease-free survival and a control computed tomography (CT) scan with no data of tumor activity. Conclusion: DT treatment should be multidisciplinary and individualized based on the behavior, location, and size of the tumor, the functional status of the limb, secondary symptoms, the risk of complications if the disease continues to progress, and the patient’s decision.
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