Introduction: Littoral cell angioma (LCA) is a new subtype of vascular tumor, which has been reported infrequently worldwide. It is associated with visceral malignancies and other immunologic conditions.
Clinical Case: We present a case of a 70-year-old Caucasian male with a 6-year history of myelodysplastic syndrome, which was investigated for splenomegaly and pancytopenia.