This case study examines a sixty five-year-old male presenting with a distended abdomen, lower back discomfort, and dyspnea on lying down, progressing over three months. Physical examination revealed multiple hard palpable lumps in the abdomen. Initial radiological evaluation indicated a connective tissue tumor, leading to a trucut biopsy revealing a spindle cell neoplasm of uncertain malignant potential. The tumor was found to be resistant to immunotherapy and neoadjuvant chemotherapy. Surgical resection unveiled multiple mesenteric tumors along the small bowel and also involving the descending colon, diagnosed as a dedifferentiated liposarcoma. Liposarcoma encompasses five histological subtypes, with dedifferentiated being the least common and comprised of poorly differentiated cells. Although liposarcomas typically originate from the retroperitoneum, the occurrence of dedifferentiated liposarcoma in the small bowel mesentery is exceedingly rare. This case report aims to underscore this uncommon occurrence, discuss diagnostic and treatment strategies, and advocate for further research into targeted therapies addressing specific immunohistochemistry markers to enhance outcomes for such patients, who generally face a grim prognosis Keywords – dedifferentiated liposarcoma , mesenteric tumors, spindle cell malignancy, mesenchymal tumors of GIT.