Clinicopathological and molecular characteristics and medication efficacy, were evaluated in 67 cases of renal epithelioid angiomyolipoma (eAML) to determine its malignant potential and prognostic markers. The immunohistochemistry characteristics of 67 patients with renal eAML were analyzed. It was determined to re-examine all patients’ FFPE slides. Around 21 patients with metastatic disease were given Everolimus 10 mg orally once a day. The RECIST criteria were used by 3 writers to evaluate the responses. The following variables were used to create a risk classification model: stage pT3 and pT4, mitotic count (2), presence of necrosis, presence of atypical mitoses, presence of severe nuclear atypia, status (SMA negative, Ki-67 10%). The epithelioid fraction averaged 85.6% (range 80-95%). Malignant features were substantially linked with immunohistochemical Ki-67≥10% and negative SMA staining (Ki-67: P<0.001; SMA: P=0.001). Analysis of survival data revealed that pT3-pT4 stage, the presence of necrosis, severe nuclear atypia, the presence of atypical mitoses, a mitotic count of more than or equal to 2, a Ki-67 more than or equal to 10%, and the absence of SMA expression was significantly linked with inferior PFS and OS (P<0.05). Among patients with renal eAML, the risk model adequately differentiated between individuals in low, intermediate, and high risk for recurrence/metastasis (AUC = 0.897) and cancer-specific mortality (AUC=0.932). After a recurrence or metastasis, 21 patients were given the targeted treatment Everolimus. About 8 of 21 patients (38.1%) had partial responses (PR) to Everolimus treatment, 9/21 (42.9%) had stable disease, and 4/21 (19%) had progressing disease (PD). The risk stratification model might identify a high risk of recurrence or metastasis from eAML. Patients with a recurrence or metastatic disease benefited greatly from everolimus-targeted treatment.