In this study, we aimed to present our surgical approach, tumor characteristics, clinical presentation and long-term follow-up results in cases of primary mesenteric fibromatosis. The data collected from 11 patients who underwent surgery due to primary mesenteric fibromatosis in our clinic between 2010 and 2019 were analyzed retrospectively. Abdominal pain, abdominal distention, and two patients (18.2%) were operated on with a diagnosis of acute abdomen in the emergency setting due to mechanical bowel obstruction in one patient. There were 11 patients in our study. Six patients were female and 5 were male. The mean age was 44.2Ã±15.8 years. Abdominal mass was detected in 5 patients (45.5%) who had complaints of mechanical bowel obstruction such as nausea and vomiting and gastrointestinal perforation in other patient. Mesenteric mass was detected in 3 patients (27.3%) with vague abdominal pain. One patient (9.1%) presented with abdominal pain and swelling of the right leg. After a mean follow-up period of 43.4Ã±28.4 months, only 1 patient (9.1%) had recurrence and required reoperation approximately 80 months after the first operation. One patient (9.1%) died of anastomotic leakage and sepsis in the first 30 days postoperatively, and other patient (9.1%) died of other reasons 1 year later postoperatively. Although mesenteric fibromatosis is a benign tumor pathologically, the main principle in the treatment of this tumor which is clinically aggressive and has high recurrence rate is wide surgical resection. Mesenteric fibromatoses have a varied clinical presentation. Radiological imaging methods helps diagnosis and planning the surgical treatment. Immunohistochemical characteristics confirms the diagnosis and differentiates from other similar tumors.