We report, to the best of our knowledge, the first case of simultaneous splenic lymphangioma and non-Hodgkin's B-cell lymphoma with liver and bone marrow involvement arising in a 69-year-old woman suffering from chronic hepatitis C infection treated with laparoscopic splenectomy followed by polychemotherapy. After 22 months from surgical treatment, the patient is alive without signs of residual disease. According to our experience, laparoscopic splenectomy followed by polychemotherapy seems to be an effective treatment for simultaneous splenic lymphangioma and non-Hodgkin's B-cell lymphoma.