Renal epithelioid angiomyolipomas (ReAML) are rare tumors (identified in less than 0,1 per thousand in general population) and represent 8% of operated angiomolipomas (AML). The diagnostic is histological, with an epithelioid cell component among the typical AML cells. ReAML are tumors derived from perivascular epithelioid cells (PEComa). There are benign PEComas, potentially aggressive PEComas and malignant PEComas. Most malignant PEComas are ReAML. There are two ReAML clinical entities, sporadic or associated to Tuberous Sclerosis Complex (TSC). ReAML are unique, localized and sporadic solid tumors of the kidney of variable size that can be revealed as classical AML with local symptoms or a complication (hemorrhage). Revelation mode is mostly radiologic. ReAML are fat-poor on CT-scan. They can be misdiagnosed with renal cell carcinoma (RCC). (One third of ReAML are malignant with a locoregional, nodal or metastatic evolution that can lead to death. ReAML treatments are multimodal depending of histology, clinical-radiological entity, evolution and the patient. Partial nephrectomy or follow-up are the benign entity treatment. Radical nephrectomy eventually followed by doxorubicine or rapamycine treatments are recommended for potentially aggressive and malignant entities.