Myxoglobulosis of the appendix is a rare and peculiar form of appendiceal mucocele. It is characterized by pearl or fish egg-like mucin globules in the lumen. We report a case of myxoglobulosis of the appendix in a 31-year-old man. Myxoglobules are composed of eosinophilic necrotic cell debris and mucin in the central nidus and lamellar structures alternating with necrotic cell debris and mucin in the peripheral zone. A stirring effect produced by vigorous appendiceal peristalsis may contribute to its lamellar growth.