目的 探讨肾血管平滑肌脂肪瘤（RAML）超声声像图的特殊表现及其生物学行为，为临床医生正确诊断RAML提供参考。 方法 收集1例侵袭性经典型RAML伴假性动脉瘤形成患者的临床资料，结合病理学特征分析其超声声像图表现及其生物学行为特点，并结合相关文献进行复习。 结果 患者，女性，60岁，因腰部不适于当地医院行CT检查时发现左肾占位性病变就诊本院。专科查体和实验室检查未见异常。超声提示左肾增大，上极囊实性肿块伴假性动脉瘤形成（源于叶间动脉）；增强CT影像提示左肾上极肾癌可能性大，瘤体内动脉瘤形成，伴左侧肾上腺受侵。行腹腔镜下左肾根治性切除术，术后病理证实为具有侵袭性行为的经典型RAML。 结论 经典型RAML可具有侵袭性生物学行为。伴假性动脉瘤形成是RAML超声声像图的特殊表现，与其他肾脏肿瘤较难鉴别。

Objective To discuss the distinctive sonographic feature and the biological behavior of renal angiomyolipoma （RAML）， and to provide the reference for the clinicians to make the accurate diagnosis of RAML. Methods The clinical data of one patient with invasive classical RAML combined with pseudaneurysm formation were collected. The sonographic appearances were analyzed in conjunction with the pathological characteristics to clarify the biological behavior of RAML， and the relevant literatures were reviewed. Results The patient， a 60-year-old female， visited the local hospital due to discomfort in the lumbar area，and received CT examination，and the CT examination results revealed a left renal mass，so the patient came to our hospital. The specialist clinical examinations and laboratory investigations were unremarkable. The ultrasound results indicated an enlarged left kidney with a cystic and solid mass at the upper pole， which featured pseudaneurysm formation （originating from the interlobar arteries）； the enhanced CT image results suggested a high probability of upper pole renal carcinoma combined with aneurysmal formation within the tumor， alongside invasion into the left adrenal gland. The patient underwent laparoscopic radical left nephrectomy， and the postoperative pathology confirmed the diagnosis of invasive classical RAML. Conclusion The classical RAML can exhibit the invasive biological behavior. The pseudaneurysm formation is a special sonographic manifestation of RAML， which can be challenging to differentiate from the other renal tumors.