Purpose: To present an uncommon clinical presentation of renal cell carcinoma (RCC) with progressive right scrotal wall varicose. Case report: A 41-year-old male patient visited our hospital due to progressive painless vessel engorgement beneath his right scrotal skin for 2 months. Physical examination revealed right scrotal wall varicose accompanied with right varicocele in his right scrotum. Ultrasound study and abdominal computerized tomography demonstrated a 9.3 cm heterogeneous tu-mor at the right kidney and extends into the renal vein. Dilation of the right gonadal vein was also noted owing to the receiving of collateral venous drainage from the right kidney which leaded to venous stasis in the right scrotum. He subsequently underwent a right radical nephrectomy, and the histopathological examination demonstrated RCC with renal vein thrombosis. His scrotal varicose veins and vari-cocele disappeared gradually in one month after operation. He has no tumor or vas-cular lesion recurrence in 2 years follow up. Conclusion and Importance: Adult right varicoceles usually link to some pathologic processes. This case report reminds further investigations are mandatory in patients with newly unset of right site scrotal wall varicose or varicocele.