The clinicopathological features of 1 case of pediatric liver epithelioid inflammatory myofibroblastic sarcoma (EIMS) with microcystic, acinar and solid structures were analyzed by light microscopy, immunohistochemistry and fluorescence in situ hybridization, and the corresponding differentiation was made based on literature review. The patient was a 3-year-old girl with a large liver space. Postoperative pathologic microscopy showed that the tumor was clearly bounded from the liver tissue, and the main body of the tumor presented an uneven microcystic or alveolar-like structure, with the cyst wall covered with polygonal or short-spindle epithelioid cells, and a large number of lymphocytes, plasma cells and tissue cells infiltrated in the cyst cavity and stroma. Local epithelioid cells were acinar to solid sheets. Immunohistochemistry: epithelial tumor cells CK18, Vim, and Desmin were strongly positive, and ALK was negative, with the Ki-67 index of about 3%. After comprehensive analysis, the patient was diagnosed as epithelioid inflammatory myofibroblastic tumor. The patient was followed up 9 months after the operation and was in good condition without tumor recurrence or other abnormalities. The special histological morphology of this case is EIMS, which has not been described in the literature, and the diagnosis is mainly based on morphological, immunohistochemical and molecular detection. Clinically and pathologically, multiple tumors need to be identified. The main treatment is surgical resection, and close follow-up is needed to understand its biological behavior and prognosis.