Primary spleen neoplasms are rare diseases. Most often we find a tumorous involvement of splenic parenchyma as an accidental finding but sometimes it is a spontaneous spleen rupture. Littoral cell angioma (LCA) is a unique benign vascular neoplasm of splenic sinus-lining cells with unknown aetiology. LCA has no soft tissue counterpart. Patients with LCA has no age and sex predilection. In 1991, Stephen Falk and his colleagues first described LCA. Since then, according to the literature, 204 publications have appeared in the last 33 years. This article presents a short review of littoral cell angioma and a Hungarian case report. In Hungary our case is the first and the only.