Desmoid tumors are benign but locally aggressive tumors of mesenchymal origin which are poorly circumscribed, infiltrate the surrounding tissue, lack a true capsule and are composed of abundant collagen. Despite their aggressive local infiltration, desmoid tumors lack a metastatic potential. However, because of this local infiltration and compression of surrounding structures, a high recurrence rate exists and in anatomic locations with restricted access to surgical resection. The clinical behavior and natural history of desmoid tumors are unpredictable and management is difficult. We present the case of this rare medical entity in a 23 year old female. Presented with a lump abdomen associated with dull aching pain since 7 months and had undergone elective appendicectomy 2 years back.Radical resection of the affected abdominal wall musculature down to the peritoneum was performed to include a peripheral margin of 3 cm of healthy tissue and the defect was reconstructed using a vicryl/prolene mesh.