A 67-year-old woman was evaluated for asymptomatic microscopic hematuria. Intravenous urography and computed tomography demonstrated an adrenal mass, but the workup for a functional adrenal tumor was negative. The mass was surgically resected, with a histologic diagnosis of adrenal ganglioneuroma. No further treatment was necessary. After the diagnosis was made, the patient reported a family history positive for neuroblastic tumors. Two of her grandchildren had presented at early ages with a ganglioneuroblastoma and a third had presented with a ganglioneuroma. This presentation is unique because ganglioneuroma, especially that of adrenal origin, is rare in the adult population, and familial cases are extremely uncommon.