Abstract. The current study reports the case of a 29-year-old female with a long-standing history of hypertension and headaches who presented to the Outpatient Clinic of The Central Hospital of Lishui (Lishui, Zhejiang, China). Abdominal ultrasound and contrast-enhanced computed tomography were performed, which showed a left renal neoplasm, prompting a diagnosis of renal angiomyolipoma or renal cell carcinoma. After a laparoscopic partial nephrectomy was performed, a number of different diagnoses were suggested by several pathologists from eight hospitals. Considering the patient's gender, age, medical history, histopathological features and immunohistochemistry, a final diagnosis of a juxtaglomerular cell tumor (JGCT) was established. The present study therefore indicates that the possibility of a JGCT should be considered when young adults present with renal parenchymatous tumors and high blood pressure. In addition, pathologists must take clinical information into account to form a precise diagnosis.