A 4-month girl presenting with a giant mass in left adrenal region and multiple hepatic nodules was hospitalized. On the basis of computed tomography, ultrasound and epidemiology we preferred the diagnosis of a left adrenal neuroblastoma accompanied with multiple hepatic metastases preoperatively. But postoperative diagnosis was hemangiomatosis characterized by synchronous presence of a giant splenic hemangioma and multiple hepatic hemangiomas. We think that it is deservedly reported because of not only its rarity but also pitfalls of preoperative differential diagnosis and management principle.