A 10-year-old boy known case of Gaucher disease about 2 years ago was admitted with nausea, vomiting and epigastric pain from 3 days before hospitalization to Mofid Children’s hospital, Tehran, Iran. On the initial examination, no abnormalities were found except for splenomegaly and brief tenderness on the spleen. In secondary paraclinical evaluations (with Ultrasound and Ct-scan), we noticed mesenteric lymphadenopathy in the patient. The lesions remained unchanged and no significant changes were observed in the clinical and laboratory evaluations of the patient during one-year follow-up period. This is a rare and benign manifestation of Gaucher disease, which is usually monitored and does not require any intervention without signs of malignancy or severe space occupying effects