We report the case of a 58-year old patient, showing a solid image in the right kidney, who underwent radical nephrectomy that revealed neoplasia, whose pathological study led to the diagnosis of kidney carcinoma associated with Xp11.2 translocation / TFE3 (ASPL-TFE3) gene fusion. The authors discuss aspects related to this lesion, such as frequency, pathogenesis, clinical presentation, histopathology and outcome, as observed in the literature.

We report the case of a 57-year old patient with complex cystic image in right kidney. Following radical nephrectomy, the pathological study established the diagnosis of renal cholesteatoma. We discuss the frequency, pathogenesis, clinical presentation, propedeutics, histological findings and proposes for intervention observed in the literature.