Background Desmoid-type fibromatosis is a low grade soft tissue sarcoma that derives from mesenchymal progenitor cells. Without characteristics of imaging, desmoid-type fibromatosis often confused with stromal tumors clinically. The disease characterized by local invasiveness and local recurrence but rare do metastasize. It can occur anywhere in the body. Most common location is the abdominal wall, nearly 30%~50% in all cases. It can also occur in the abdominal cavity, with a low incidence. At present, all types of desmoid-type fibromatosis follow the treatment consensus of extra-abdominal type. However, because of the difficulty in preoperative diagnosis, sometimes it is hard to choose the appropriate treatment. Case presentation We describe the case of a 22-years-old woman, who was diagnosed with an abdominal mass . She was a healthy individual who was incidentally diagnosed with abdominal mass. The mass was under the suspicion of gastrointestinal stromal tumors in a local hospital, according to the result of MRI scan. A CT scan of her abdomen showed a huge hypoechoic mass in the hepatic. Under misdiagnosis as hemangioma of liver in our hospital, she underwent a laparoscopic resection of the tumor . Postoperatively, the tumor was confirmed to be a desmoid-type fibromatosis by patholgical and immunohistochemical findings. Conclusions In conclusion, due to the high risk of recurrence, the diagnosis before treatment is the most important part in the management of desmoid-type fibromatosis. Even if it is rare, DF should also be included in the differential diagnosis. Treatment needs to be evaluated according to the location of the lesion. For lesions on the body surface, pathologic examination will be safer and more useful. Surgery is still one of the best choice for intra-abdominal desmoid-type fibromatosis patients. Meanwhile, in order to change the operation in time, the clinicals should keep a rigorous attitude in surgery, if preoperative diagnosis is difficult.