Desmoid tumors, characterized by aggressive local infiltration of surrounding tissues, are uncommon benign neoplasms with no metastatic potential , that occasionally may attain large size. We report a case of a 37-year-old woman with an abdominal wall desmoid tumor that appeared and grew rapidly during her pregnancy, diagnosed by trucut core biopsy. Complete surgical excision of a 20x16cm in size tumor and immediate reconstruction with mesh was performed in the postpartum period. She had no postoperative complications and no recurrence at 2-year follow-up. Optimal management of large abdominal wall desmoids during pregnancy has to be individualized, with wide surgical excision remaining the treatment of choice.