Abstract
A 44 year-old female, known to have Li-Fraumeni syndrome, was discovered to have a retroperitoneal mass on surveillance ultrasound. Computerized tomography revealed a 2.0 cm non-cystic, enhancing soft tissue mass abutting the postero-superior margin of the right kidney. She underwent radical nephrectomy, adrenalectomy and en bloc resection of the tumor. Pathology showed a grade I, well-differentiated retroperitoneal liposarcoma with negative surgical margins. She recovered well from surgery and continues to remain recurrence free at 12 months based on serial CT imaging.