BackgroundCastleman's disease or angiofollicular lymphoid hyperplasia is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. The disease can present at any extra thoracic site with lymphoid tissue such as retroperitoneal, mesentery, axilla, and pelvis. Hepatic localization castleman disease is very rare in children. Herein, we reported a case of Castleman's disease arising from the lymph node in hilum of liver.Case presentationA 5 -year-old girl with chief complaint of abdominal pain for two months which exaggerated in last three days was referred to the hospital. On routine physical examination, only a generalized abdominal pain was noticed. Routine laboratory investigations and Chest X-Ray were normal. Abdominal Sonography revealed a 3.7 × 3.1 cm solid mass in the hilum of the liver. On the MRI images, a lobulated mass in the portal hepatic associated with mass effect on the portal vein was visible. Histological examination revealed expansion of mantle zone in lymphatic nodules accompanied by burnt out germinal centers. This pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease. The patient underwent a laparotomy. The patient had an uneventful postoperative course.ConclusionThis pattern was matched with the diagnosis of the hyaline-vascular type of Castleman disease.