ObjectiveTo summarize the effect of the multi-disciplinary treatment (MDT) for a patient with giant pancreatic cystic lymphangioma.MethodsThe clinical data of a patient with pancreatic lymphangioma admitted to the Affiliated Hospital of North Sichuan Medical College in March 2020 was retrospectively analyzed, then reviewed the literatures, and summarized the preoperative diagnosis, treatment, and management of perioperative period and long-term period after surgery of this rare disease.ResultsAfter admission, the patient was diagnosed as pancreatic tumor by various imaging and laboratory examinations. The nature was unknown. After discussion by MDT, it was decided to undergo a total pancreatectomy (TP). After the operation, drugs and diet were given to regulate blood glucose. The patient’s tumor disappeared after the operation, and no tumor recurrence was found in the three months after discharge.ConclusionsPancreatic lymphangioma is rare and lacks a clear diagnosis and treatment plan. The MDT mode can bring a clearer diagnosis and more effective treatment for it.