A 58-year-old, otherwise healthy man presented with a sudden onset of watery diarrhea. A pseudomembranous colitis due to antibiotics was identified as the cause of the diarrhea. Enlargement of the spleen was detected during the evaluation. The enlarged, plump spleen (20 cm long, 7.1 cm wide) had multiple nodules that differed in size from 1 to 8 cm. Neither clinical nor other symptoms of an underlying malignant disease could be detected. Because the signs were of little diagnostic value we arranged a splenectomy, which showed a littoral cell angioma (LCA) to be the cause of splenomegaly. In addition to the case report, we have reviewed the literature, clinical manifestations, differential diagnosis, special gross and microscopic pathological findings, and the location of this benign vessel tumor in the pathology of the spleen.