A 14-year-old girl with intermittent headache, palpitations, tachycardia, and diaphoresis.

Pheochromocytomas are neuroendocrine tumors originating from chromaffin cells.  Most arise from the adrenal medulla, while approximately one out of ten cases originate from the extra-adrenal paraganglia. These catecholamine-secreting tumors cause a broad spectrum of symptoms and may resemble various clinical disorders. Their clinical picture varies greatly, from the classic triad of headaches, diaphoresis and palpitations, to asymptomatic tumors discovered incidentally on imaging. We present the case of a 14-year-old girl with pheochromocytoma of adrenal origin and grade IV hypertensive retinopathy and review the clinical picture, follow-up examination, molecular findings and outcome of the 11 reported cases of pheochromocytoma and hypertensive retinopathy in children.