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. Radiological and histopathological examinations revealed an LCA and an open splenectomy were performed. The patient had an uneventful post-operative recovery. Conclusion: LCA is a rare tumor, with atypical presentation often associated with other malignancies or immunologic conditions. Diagnosis is challenging, and so far, splenectomyis the gold standard treatment.