Renal angiomyolipoma (AML) is a benign mesenchymal neoplasm driven by biallelic inactivation of TSC genes. Regional lymph node involvement is rare in classic AML and may raise concern for malignancy or metastatic disease.

We present a case of a 43-year-old woman with a large, complex renal mass measuring 23 cm and extending across the midline, radiologically concerning for either AML or liposarcoma. The patient had no clinical or genetic evidence of tuberous sclerosis. Due to the risk of hemorrhage, arterial embolization was attempted but was unsuccessful, necessitating right radical nephrectomy. At surgery, several retroperitoneal lymph nodes were noticeably enlarged. An intraoperative frozen section revealed only classical AML component without any epithelioid elements, providing reassurance to minimize the extent of lymph node dissection. Histopathologic examination confirmed classic AML with involvement of nineteen regional lymph nodes. No evidence of epithelioid morphology or malignancy was identified. The patient recovered uneventfully and remains disease-free on follow-up.

Lymph node involvement in AML should not be misinterpreted as metastasis. According to current WHO consensus recommendations, such findings are best interpreted as multicentric tumor spread rather than metastasis. Awareness of this phenomenon is critical for surgical decision making, to avoid overtreatment and misclassification as renal cell carcinoma (RCC) metastasis.