Littoral cell angioma is an uncommon primary vascular neoplasm of the spleen. It frequently follows a benign course, but cases with aggressive behavior have been described. We present a case of this rare disease highlighting radiological examinations showing a rapid increase in the size of the spleen as well as an increase in the number and size of existing nodules. We also discuss the relationship of this disorder to chronic infections and its possible association with Epstein Barr virus.
