Renal cell carcinoma (RCC) accounts for 90–95% of all kidney neoplasms, with 25–30% of patients presenting with metastases at diagnosis. Common metastatic sites include the lungs (45%), bones (30%), lymph nodes (22%), liver (20%), and adrenal glands (9%). Vaginal metastasis is extremely rare, with fewer than 100 cases reported. It may occur via retrograde venous flow through the ovarian vein or Batson’s plexus and may initially present with gynecologic symptoms or abnormal cytologic findings.

A 64-year-old woman with controlled hypertension presented with a 1–2 month history of painless gross hematuria. Imaging revealed a large left renal mass with renal vein thrombus and suspected pancreatic tail involvement, along with bilateral pulmonary nodules. She underwent left open radical nephrectomy and renal vein thrombectomy, with pathology confirming ISUP grade 4 clear cell RCC with rhabdoid differentiation. Staging was cT3aN0M1. Gynecologic evaluation revealed irregular vulvovaginal neogrowths; biopsy confirmed metastatic RCC. Days later, she sustained a pathological right femoral shaft fracture, confirmed radiographically and managed with open reduction and internal fixation. The patient is currently receiving cabozantinib and remains under close follow-up.

Although vaginal metastases from RCC are rare, they should be considered when atypical gynecologic symptoms or malignant cytology occur, especially in patients with a known RCC history. This case illustrates the diagnostic complexity and highlights the need for a multidisciplinary approach involving urology, gynecology, pathology, radiology, and orthopedic oncology.

We report an unusual case of metastatic clear-cell RCC with rare vaginal and uterine metastases, and subsequent femoral shaft fractures. This case emphasizes the importance of a multidisciplinary approach for diagnosis and management, particularly in patients presenting with atypical gynecologic or orthopedic symptoms.