Abstract
Desmoid tumors or aggressive fibromatosis are locally aggressive benign tumors. These arise anywhere in the body but are commonly seen in the anterior abdominal wall. The main treatment choices are continuous surveillance, adjuvant chemotherapy, surgery, and postoperative chemotherapy. However, where needs arise, surgery may be done, specifically wide local excision with adequate clearance. In our case report we show a case of a male patient presenting with umbilical hernia and an incidental discovery of a desmoid tumor that was closely proximate to the herniated sac. The plan of action planned were sac excision followed by tumor excision and then anatomical repair, that is, umbilical herniorrhaphy and postoperative chemotherapy.