Renal cell carcinoma (RCC) is rarely diagnosed during pregnancy and its management represents a real challenge. As the symptomatology might mimic other common pregnancy-related disorders, the diagnosis is often delayed. RCC should be considered in women of childbearing age who present with recurrent or refractory urinary tract symptoms, flank pain, or a palpable mass. Ultrasound appears to be the imaging procedure of choice followed by the magnetic resonance imaging for evaluating the urinary system in pregnant women. The probability of cure is directly related to the stage or degree of tumor dissemination. Surgical resection is the mainstay of treatment.