Varicocele represents the main cause of male infertility. Right-sided varicocele is rare and can be due to renal malignancy or a venous abnormality. The most common anomaly of the inferior vena cava (IVC) is interruption of IVC with azygos continuation, which is recognized as an uncommon congenital anomaly. The prevalence of the interruption of IVC is less than 0.3% in the healthy population. We describe the case of a 26-year-old man who had right varicocele because of a right-sided IVC with a retro-aortic left renal vein and azygos continuation. The right and left IVCs received the right and left common iliac veins, respectively, and the left renal vein crossed posteriorly to the aorta and joined the right IVC. The right IVC continued cephalad as the azygos vein within the retrocrural space. Isolated right-sided varicoceles are uncommon, but practitioners should be aware of such a condition. In case of a venous anomaly, clinicians should aware of the association with other important clinical presentations.