We report a case of Paget sarcoma of the left superior pubic ramus and disseminated metastatic disease in a 70-year-old man. Paget disease of the left hemipelvis with malignant degeneration in the region of the left superior pubic ramus was initially diagnosed on radiographs. Subsequent CT, MRI, PET/CT imaging, and CT-guided biopsy confirmed the diagnosis and showed extensive left-sided pelvic and abdominal lymphadenopathy with widespread metastatic disease to liver, spleen, and lungs.