Mesenteric lymphadenopathy is a rare manifestation of Gaucher disease (GD) with only 26 cases reported worldwide and its outcome remains largely unknown.In this manuscript,we described a 17-year-old girl with GD who has been treated with standard enzyme replacement therapy (ERT) for 16 years.The follow-up of her mesenteric lymphadenopathy began 13 years ago,which is one of the longest follow-up for this condition worldwide.