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. Copyright (C) 2012 S. Karger AG, Basel