Renal cell carcinoma (RCC) is an extremely rare and fatal tumor for women of childbearing age. Consequently, the cases published in the literature are limited and medical experience with therapeutic management of newly diagnosed RCC in pregnant women is poor. We report our clinical experience with a renal tumor diagnosed in first-trimester pregnancy complicated in postoperative by an inferior vena cava thrombosis. Further reporting of such a case should help to collect more data in purpose to establish a clear, systematic and evidence guide-ligne in front of this unique situation.