This case describes a 40-year-old man with abdominal pain, nausea, and vomiting, who was found to have a cystic lesion in the tail of the pancreas. Distal pancreatectomy and splenectomy revealed a primary epidermoid cyst of the spleen lying in the substance of the tail of the pancreas. This represents the only report of a splenic cyst in an ectopic spleen that could be found in a search of the world literature.