Banti's disease is a condition where congestive splenomegaly can be observed in the absence of intrahepatic or extrahepatic obstruction. The diagnosis is established by splenectomy, but it is necessary to exclude liver diseases or portal vein obstruction before surgery. The advanced stage of Banti's disease may be complicated by upper gastrointestinal haemorrhages; so splenectomy has both diagnostical and therapeutical benefits. Primary lesions of the small splenic arterioles are one of the offered explanations. A patient with no intrahepatic or extrahepatic obstruction, is described. We also excluded diseases which might be complicated by splenomegaly, part of them after splenectomy. Cytogenetics showed normal female pattern. The histologic examination revealed no liver disease, but the spleen was congestive with reduced lymphoid tissue. Immunohistologically, the reduced spleen white-pulp nodules were composed of polyclonal B-cells and T-cells in a normal distribution, discarding indolent lymphoprolipherative disorder. Over one year after splenectomy the patient had no trouble. In conclusion, we believe that Banti's disease is a condition clearly separated from Banti's syndrome. We also believe that splenectomy is the treatment of choice.