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