A case of an inflammatory pseudotumor of the spleen is presented. A 43-year-old woman was hospitalized for a detailed examination due to pancytopenia, which was diagnosed during an examination related to left upper quadrant abdominal pain. Physical examination revealed a 2 to 3 cm splenomegaly. Reticulocyte count was 4% and erythrocyte sedimentation rate was 55 mm/h. No other important findings were noted, except for an evident increase in myeloid series and megakaryocytes (more evident in erythroid series) on bone marrow aspiration and biopsy. Computed tomography (CT) scan revealed splenomegaly and a centrally located hypodense lesion with a 92 X 86 mm axial diameter in the spleen. With a hematologic malignancy prediagnosis, a splenectomy was performed on the patient because of pancytopenia and splenomegaly. An intrasplenic, centrally located, well-limited, capsulated lesion (9.5 x 11 x 10 cm in diameter) was discovered on macroscopic examination of the material. A cellular infiltration area was seen on microscopic examination. The spleen capsule was mildly fibrotically thickened. The lesion that separated from the spleen tissue consisted of diffusely proliferated fusiform fibroblasts, heterogenous inflammatory cells consisting mainly of plasma cells, lymphocytes, sparse neutrophils and vascular elements. No granuloma or multinuclear giant cells were detected. Pancytopenia improved on follow-up. The patient followed up for two years, is now healthy and has no complaints.