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A littoral cell angioma (LCA) is a primary vascular tumor of the spleen, that can have malignant potential and may present association with other malignancies. This is a case of LCA that was discovered incidentally in a 79-year-old woman who presented with a polycythemia at the time of consultation. The neoplasm was evaluated by ultrasound and computed tomography. The patient underwent a splenectomy that revealed LCA by pathological evaluation. The post-operative outcome was favorable with no complications or recurrent disease. This case presentation, clinical, radiographic, and pathological features of an uncommon splenic tumor can be studied in order to advance our knowledge in our understanding of LCA.