Desmoid tumor, recently renamed as desmoid type fibromatosis, is an uncommon neoplasm. The mesentery is the usual origin of intra-abdominal desmoid-type fibromatosis and despite the fact there is no metastatic potential, it can infiltrate adjacent organs. There etiology remains unknown, preoperative diagnosis is difficult and differential diagnosis includes among other gastrointestinal stromal tumor (GIST). In resectable tumors, the mainstay of treatment is surgical resection with at least clean microscopic margins. We present a case of a 51-year lady who presented with a 6.2 x 4.5 x 3.3 cm neoplasm in the anatomic area of the greater gastric curvature, the splenic hilum and the tail of the pancreas that was diagnosed as GIST on the CT scan. The patient was submitted to laparoscopic excision of the tumor and histopathological examination revealed desmoid type fibromatosis of the splenic hilum infiltrating the spleen, pancreatic tale and greater gastric curvature. According to the authors' knowledge, this is the first reported case of a sporadic splenic desmoid tumor, which has been treated successfully by laparoscopic en block resection.