Myxoglobulosis is a rare morphologic variant of appendiceal mucocele characterized by intraluminal mucinous globules of the appendix. Most reported cases have presented clinically as an acute abdomen or as an incidental laparotomy or autopsy finding. We report a case of myxoglobulosis in a 32-year-old man who presented with an extra-appendiceal mass following a 10-year symptomatic course. Laparotomy disclosed a pericecal collection of opaque, white globules originating from a perforated appendix walled off by fibrous adhesions. The globules exhibited some histologic and staining properties at variance with those described in previous reports. These findings suggest that myxoglobulosis may be more heterogeneous pathogenetically than the distinctive gross appearance of the lesion would indicate.