This article reports the case of a 22-year-old woman with right renal angiomyolipoma (AML) and inferior vena cava thrombus. Laparoscopic right nephrectomy and thrombectomy were performed. To the authors' knowledge there have been only 46 reported cases of renal AML with endovascular extension and this is the first case to be completely removed by a laparoscopic approach. Laparoscopic management of this kind of tumour is feasible in spite of the vascular involvement. The centre's experience and enlargement of the tumour are key points for this approach.