Desmoid tumors are fibromatous lesions that are the result of abnormal proliferation of myofibroblasts. Despite its benign microscopic appearance and non-metastasizing behavior, tumor infiltrates surrounding tissues and has a high risk of recurrence. Pregnancy-associated desmoid tumors are very rare and optimal management of this tumor is not well established. The authors report a case of a 31-yearold pregnant woman with a large desmoid tumor, which increased rapidly in size and caused worsening symptom of dyspnea. The tumor was successfully removed during a caesarian section, which resulted in an anterior abdominal wall defect. Reconstruction of the abdominal wall defect was performed with a polypropylene mesh. The postoperative recovery of the patient was uneventful. After a follow- up of 44 months, the patient was found to be well and there was no evidence of local recurrence. The authors also reviewed the literature on the world’s experience with this tumor and its management during pregnancy. Twelve desmoid tumors arising during pregnancy were reported in the existing literature; the managements were varied and has yet to be defined.