Arterial fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic, occlusive condition of the systemic arteries, most frequently affecting renal arteries. Renal cell carcinoma (RCC) might be associated with arterial hypertension; however, there are no data in the literature regarding the relationship between RCC and associated renal artery changes. We analyzed a consecutive series of 57 (35 male and 22 female) patients aging from 35 to 79 years (mean 58.9 years) who underwent nephrectomy due to RCC in the year 2003. The patients had RCC measuring from 2 to 16 cm (mean 7.1 cm). Specimens were routinely fixed, embedded in paraffin, cut, and stained with hematoxylin and eosin, Mallory trichrome method, and orcein. Renal arteries of 26 patients (20 male, 6 female) showed no changes. In these patients, RCC measured 2.5–11 cm in largest diameter (mean 6.6 cm). In 24 patients (10 male, 14 female), renal arteries showed FMD. RCCs in these patients measured between 2 and 16 cm (mean 8.0 cm). Seven patients had atherosclerotic changes in renal arteries. In this series, FMD was found in a significant proportion of patients with RCC, mainly in women. The cause of such changes and their relationship with RCC and systemic hypertension should be further analyzed.