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Objective:   To report a hypertensive and systematically pigmented female with primitive neuroectodermal tumors.   Clinical Presentation and Intervention:   A female patient presented with a complaint of right flank pain. She had a right renal space-occupying lesion, underwent right radical nephrectomy, and returned to normotensive postoperatively. The pathological examination identified typical primitive neuroectodermal tumor histology. During a 60-month follow-up period, she remained normotensive and demonstrated normal renal and adrenal functions.   Conclusion:   Early diagnosis and definitive surgery led to the patient’s long-term survival.

Renal Primitive Malignant Tumor with Endocrine Activity