Introduction. Inflammatory myofibroblastic tumors (IMT) of the spleen are rare neoplasms and only little is known about the origin and behavior of these tumors. Here we report the case of a 37-year-old woman with an atypical spindle cell neoplasm showing features strongly suggesting an IMT of the spleen with hepatic metastasis. Methods. A 37-year-old patient had been complaining about pain in the left upper abdomen for the last two months. A CT scan revealed a tumor mass in her spleen and liver. After complete staging, a splenectomy and atypical liver resection of segments VII and VIII were performed. Literature was screened for similar cases and existing further literature. Results. A R0 resection was achieved. Histological analysis showed a multinodular infiltration of the spleen by an atypical mesenchymal neoplasia. Immunohistochemically there was an expression of histiocytic markers (CD4, CD68) as well as smooth muscle cell markers (SMA, H-Caldesmon) in the tumor cells. A diagnosis of an atypical spindle cell neoplasm showing features most suggestive of an IMT was rendered. Conclusion. Synchronous hepatic metastasis of an IMT of the spleen is a rarity. Therefore no experience in the treatment of these tumors exists. Fibroblastic reticular cell tumor is a differential diagnosis, but differentiation of these two entities is difficult.