Littoral cell angioma (LCA) is a rare primary vascular neoplasm of the spleen. A 54-year-old man was referred to our emergency department for abdominal pain. A CT scan showed multiple round hypodense lesions of various sizes throughout the spleen. The spleen was increased in volume. An MRI confirmed the lesion with a suspect of multiple angiomas vs. amartomas. The haematologists excluded any haematological disease. After a collegial discussion, we decided to perform laparotomic splenectomy. Histologically, the multiple lesions consisted in anastomosing vascular channels lined by plump cells. There was an increased number of dysmorphic megakaryocites inside the splenic parenchyma and along the tumour's border, known signs of extramedullary hemopoiesis, whose etiology in our patient was unexplained. To the best of our knowledge this is the third description of the association between littoral cell angioma and extramedullary hemopoiesis. LCA is a rare primary splenic vascular tumour that originates from the splenic littoral cells. The diagnosis of littoral cell angioma is confirmed histologically and on immunohistochemistry. This case report underlines the rarity of this type of benign splenic neoplams, but since the malignant potential of LCA, we recommend close clinical follow- up of patients with LCA of the spleen.