Lymphatic malformations--previously called "lymphangiomas"--emerge due to an abnormal development of the lymphatic channels, with obstruction of the lymph flow and formation of lymphatic cysts. These cystic masses are frequently located in the neck and axillary region (95%). A pancreatic localization is extremely rare in children. Pancreatic lymphatic malformations should be distinguished from other cystic pancreatic masses. For those located in the head of the pancreas, a pancreaticoduodenectomy is proposed. We report the case of a 13-month-old child with a lymphatic malformation on the pancreas head, resected through a pancreaticoduodenectomy. A persistent chylous drainage followed, causing immunitary impairment and an overwhelming sepsis. A non surgical approach to this lesion is discussed, and a literature review is made.