A 70-year-old man with a history of chronic lymphocytic leukemia (CLL) underwent FDG PET/CT scan, which revealed a large polypoid soft tissue lesion in the esophagus with peripheral FDG avidity. An endoscopic biopsy revealed inflammatory changes with scattered CLL cells. The final histopathology demonstrated an 18-cm long and 4-cm wide giant fibrovascular polyp that was removed in 2 pieces. The polyp was composed of atypical adipose tissue with scattered giant cells and spindle-shaped cells as well as foci of CLL. Mouse double minute 2 homolog amplification was noted by fluorescence in situ hybridization diffusely in the giant polyp consistent with well-differentiated liposarcoma in a giant fibrovascular polyp.