A case- review describing a desmoid in a young female. The tumor originated at the tendinuous attachment of the m. rectus abdominis on the pubic bone, which had had two relapses. During the third--so far the latest--procedure it required left-sided hemipelvectomy with resection of the rectum and a part of the vagina. The patient was instructed about the serious character of the disease, the requirement for the radical resection and about the requirement for a careful long-term follow-up of the patient after the procedure.