BACKGROUND Blood pressure (BP) is not routinely screened in children in clinical practice. Renal cell carcinoma (RCC) is a rare cause of renal hypertension and accounts for less than 0.3% of all childhood tumors. The clinical manifestation of hypertension in children requires a high index of suspicion, as does RCC, which can have many different manifestations. CASE REPORT We report the case of a 10-year-old girl with 1-year history of persistent symptoms of recurrent episodes of headache and excessive sweating and a 6-months history of weight loss and loss of appetite. She was repeatedly managed as having malaria in the center where she was referred, without recovery. Persistent high BP was discovered in our center, which ranged between 180/120 and 200/120 mmHg. This was not controlled by 3 different classes of drugs. Abdominal ultrasonography showed a right kidney with a well circumscribed lower pole mass with internal echoes, compressing the pelvicalyceal system. Abdominal computed tomography revealed a huge, circumscribed, expansile, isodense mass arising from the renal cortex in the lower pole of the right kidney. Intraoperative findings included a mass seen at the lower pole of the right kidney with histology diagnosis of RCC. Other laboratory tests were normal. To date, the patient remains normotensive and symptom-free after nephrectomy. CONCLUSIONS The nonspecific clinical manifestation found in this case show the need for hypertension screening in children. The resolution of symptoms after nephrectomy confirms RCC as the underlying cause of symptoms, making this case a unique presentation.