Renal cell carcinoma is rarely diagnosed during pregnancy and its management represents a real challenge. Pregnancy demands special consideration in terms of diagnostic evaluation and management, particularly during the second trimester. We report a patient undergoing left radical nephrectomy using a thoracoretroperitoneal approach at 22 weeks' gestation. Histological analysis revealed a pT2R0G2 chromophobic renal cell carcinoma. Furthermore, we review the sparse literature available.