Due to the late onset of symptoms, retroperitoneal liposarcoma are often diagnosed in advanced stages when adjacent organs have been infiltrated and the tumours have reached extensive sizes. Surgery remains the first choice of therapy. We report on the primary resection of a 45-kg liposarcoma that was removed en-bloc including the left kidney and descending colon with -tumour-free margins. Nine months later, the follow-up revealed a right-sided recurrence of the tumour, which was surgically removed including the right ureter. Since then, the patient has been without any signs of tumour recurrence or metastases. This report demonstrates that even extreme-ly large tumours can be removed safely and that the size is not a contraindication for primary surgical treatment. Local recurrence is common as seen in our case, and occurs even after R0 resection up to 10 years after the first operation. Recurrences should be surgically removed as this is the only treatment which has been shown to increase survival in even R1 and R2 situations.