Hereditary spherocytosis (HS) is the most frequent cause of congenital hemolytic anemia. Extramedullary hematopoiesis (EMH) mimicking a mass may develop in the lymph nodes, kidneys, pleura, mediastinum, adrenal gland, and in particular the spleen and liver. Other than EMH, B-cell lymphoma, acute lymphoblastic leukemia, and pancreatic schwannoma cases were reported in patients with HS. We present a 13-year-old female patient with HS and ganglioneuroma in the adrenal gland. This association is probably coincidental; however, with increasing cancer cases in HS and the genetic studies being made, this association will be clarified.