Inflammatory pseudotumor of the spleen is a benign tumorous lesion of unknown etiology and pathogenesis that has been described in only a few cases in the literature. Recognition of this rare entity is important, as the clinical manifestations and imaging features could be indistinguishable from a lymphoproliferative disorder or another malignancy of the spleen. We report a new case and review the clinical presentation, laboratory findings, pathological and immunohistochemical studies, treatment, and prognosis of the previously reported cases of inflammatory pseudotumor of the spleen.