Previously reported ESC RCC in young patient now showing to harbor TSC mutations. This tumor corresponds to ESC RCC #4 of reference 5, a 5.0 cm renal tumor in a 25-year-old male with a history of “bladder cancer” as a child. This tumor was solid (not cystic) throughout (A, B). The neoplastic cells had abundant eosinophilic cytoplasm, well defined cell borders, and prominent granular cytoplasmic stippling, characteristic of ESC RCC. This neoplasm demonstrated two TSC2 mutations.