ATRX and neoplasm: This case demonstrates the value of NGS in screening actionable molecular targets for rare malignant reninoma; olaparib yields favorable local control for ATRX-mutated malignant reninoma based on synthetic lethality, while the differential response at metastatic sites underscores the need for exploring combination therapies to overcome tumor heterogeneity and drug resistance, providing a clinical reference for precision treatment of this rare disease.