Abstract: Objective: To study pathomorphologic characteristics of spleen Gaucher disease to improve the understanding,diagnosis and treatment of it,and to provide more information for clinical diagnosis.Methods: H-E,PAS staining and immunohistochemistry technique were used for the tissue sample.And literature concerned were reviewed.Results: The patient presented with enlarged liver and spleen.Gaucher cells had abundant cytoplasm containing distinct striations and were strong positive for PAS.Conclusions: Gaucher disease should be considered for the patient with chronic megalosplenia.Differential diagnosis should be made among diseases with splenomegalia.