Objective: Objective: To review the clinicopathologic characteristics, diagnosis and treatment of splenic inflammatory myofibroblastic tumor (SIMT). Methods: Clinical data of SIMT patients including 4 cases admitted in Guangzhou General Hospital of Guangzhou Military Command and 68 cases extracted from literature reports were collected. The main clinical manifestations, imaging features, pathological diagnosis, treatment and results of the patients were analyzed and summarized. Results: The majority of the patients were male adults, left upper abdominal pain was the initial and main symptom, which was often accompanied with other systemic symptoms such as fever, fatigue, weight loss and anemia, and most of the tumors were solitary and localized with clear margins as shown by imaging examinations. B-ultrasonography showed heterogeneous echogenic masses in the spleen, with absent or barely visible color Doppler flow; CT scan showed homogeneous or heterogeneous low-density lesions in the spleen, some of which had no obvious enhancement in any contrast-enhanced phase, and most of which had slight enhancement in the arterial phase, and continuously increased enhancement in the venous and delayed phase, with the density lower than or similar to that of the spleen, and more evident change in the rims; lesions typically showed low signal on both the T1WI and T2WI in MRI imaging, most of which demonstrated no enhancement during enhanced scanning, some of which had slight enhancement in arterial phase, and continuously increased enhancement in venous and delayed phase. Hyperplastic spindle cells, chronic inflammatory cell infiltration, and collagen fibril formation were seen under microscope; immunohistochemical staining was positive for vimentin, SMA, Desmin and CD68. All the 72 patients underwent splenectomy, and followed-up ranged from 4 months to 14 years; recurrence occurred in one case 2 years after operation, and hepatic metastases occurred in one case 4 years after operation. Conclusion: SIMT is a rare borderline mesenchymal tumor, with no clinical characteristics, and can be easily misdiagnosed as malignant tumor, for which, imaging examinations have certain diagnostic significance, and diagnosis relies on immunohistochemical and pathological examination; splenectomy is the effective treatment method and prognosis is favorable, but long-term postoperative follow-up is necessary.