Adrenal adenoma with myelolipoma is extremely rare in pediatrics. Although the tumor is usually asymptomatic, sometimes it may result in serious manifestations. A 16-year-old patient was diagnosed with severe hypertension associated with a right adrenal mass. The laboratory work-up was inconclusive of the nature of the tumor. Plasma and urinary hormonal studies were not diagnostic. Magnetic resonance imaging (MRI) of the brain and meta-iodobenzylguanidine (MIBG) scanning were normal. MRI of the abdomen showed a heterogeneous adrenal mass 4.2x3.3 cm. Laparoscopic resection of the mass was done, and the pathology revealed an adrenal adenoma with myelolipoma. After tumor resection the hypertension resolved, and within 1 month the patient was off medications. At 2-year follow-up the patient's blood pressure remained normal at 120/73 mmHg. This is a case in which an adrenal adenoma with myelolipoma, a benign and usually asymptomatic tumor, presented as severe hypertension resolving with surgical resection of the tumor.