Tumors with TFE3 rearrangement include PEComa with TFE3-rearrangement, Xp11-associated renal cell carcinoma (now reclassified as MiT family translocation renal cell carcinoma), melanotic Xp11 neoplasm, and alveolar soft part sarcoma [1,3]. The gene discussed is TFE3; the disease is hereditary clear cell renal cell carcinoma.