Papillary Renal cell carcinoma is the second common histologic subtype of renal malignancy. Hereditary Leiomyomatosis and renal cell carcinoma are syndromes predisposing to papillary renal cell carcinoma type 2 along with leiomyoma uterus and skin. The prevalence of HLRCC is 1 in 200000. We present a case of HLRCC presenting with skin leiomyoma and renal cell carcinoma. This papillary renal cell carcinoma in HLRCC is associated with poor prognosis. Early and prompt excision of Renal mass and lymph nodes is the only treatment. Adjuvant treatment is not well established and not available for the common man in our part of the country. Post-surgical follow up and patient education is essential.
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