{Reference Type}: Case Reports {Title}: Huge mesenteric fibromatosis presenting with intestinal perforation and acute diffuse peritonitis: a case report. {Author}: Xu Y;Duan Z;Hu W;Zhu K;You J;Abe M;Chen P;Zhang Q;Zong L;Xu Y;Duan Z;Hu W;Zhu K;You J;Abe M;Chen P;Zhang Q;Zong L; {Journal}: Transl Cancer Res {Volume}: 9 {Issue}: 9 {Year}: Sep 2020 {Factor}: 0.496 {DOI}: 10.21037/tcr-19-1151 {Abstract}: Mesenteric fibromatosis is a locally invasive myofibroblastic proliferation and rarely metastasize to other organs. Hollow organ perforation and acute diffuse peritonitis caused by mesenteric fibromatosis rarely occurred. Here we report a case of huge mesenteric fibromatosis who complained a paroxysmal epigastric pain, and CT scan showed a huge mass, pneumoperitoneum and ascites. An urgent laparotomy showed an intro-abdominal mass and perforation locating at the jejunum. Postoperative histology confirmed it to be mesenteric fibromatosis. With one-year follow-up, the patient had no recurrence. We wish to share our treating experience of this interesting case because it did not belong to a typical type but presenting with acute diffuse peritonitis, and total resection and R0 margin is a key to treat acute case. This atypical one has not been reported in the literature till now.