Two cases of primary renal artery dissection in Caucasian males, aged 40 and 37 years, respectively, are presented. In both subjects, the clinical picture disclosed renal infarction with transient deterioration of renal function, microhematuria and proteinuria. In 1 patient, a slight, temporary increase in blood pressure was also observed. Both men experienced contralateral relapse 12 days and 7 years, respectively, after the first episode. The diagnosis was based on characteristic (case 1) or suggestive (case 2) arteriographic findings. In 1 subject, control arteriography suggested some progression of the lesion. Both patients were treated conservatively. A comparison with 143 cases previously reported worldwide emphasizes some aspects of this ‘not-so-rare’ ent