Lymphangioma is a benign form of neoplasm arising from the lymphatic system. It occurs as a result of congenital malformations of the lymphatics leading to the obstruction of local lymph flow and the development of lymphangiectasia. Lymphangiomas are common in pediatric patients, in the soft tissues of the neck and the axillae, but lymphangioma of the pancreas is extremely rare, accounting for less than 1% of these tumors. It occurs more frequently in females and is often located in the distal pancreas. Although extremely rare, cystic lymphangioma of the pancreas should be taken into consideration as a differential diagnosis of pancreatic cystic or retroperitoneal lesions, especially in women. Herein, we report on a case of cystic lymphangioma of the distal pancreas in a 37-year-old woman who was treated with complete surgical resection with a review of the literature.