Aggressive breast fibromatosis (referred to as a desmoid tumor) is a rare, locally invasive, non-metastasizing tumor with high recurrence rate. The therapeutic modalities range from surgery and radiotherapy to medical treatments. However, the optimal treatment is controversial, especially in a situation of repeated recurrence. Here, we present a case of a patient with aggressive breast fibromatosis with multiple recurrence after surgeries, who underwent high intensity focused ultrasound (HIFU) treatment effectively without side effects. To our knowledge, this is the first reported case of HIFU treatment in aggressive breast fibromatosis, which indicates that HIFU might be a novel, promising modality for this rare disease.