Aggressive fibromatosis is a rare and benign tumor of soft tissues, locally invasive but never metastasizes. This proliferation arises in musculoaponeurotic structures. It is more common in young fertile women due to hormonal influences. Radical resection with free margins is the key to an effective outcome. We present here the case of a 47-year-old woman, without medical history, who was referred to our hospital for a painful swelling of the left iliac fossa, diagnosed with aggressive fibromatosis of the anterior abdominal wall based on radiological and histological findings. She underwent an excision of the mass with free margins followed by reconstruction of the musculo-fascial defect. After a follow-up of 12 months, there was no evidence of recurrence. Management of aggressive fibromatosis remains a challenge for surgeons. Complete excision of the tumor followed by reconstruction of abdominal wall defect is the first choice of treatment.