The designation of clear cell/lipid-rich refers to an unusual variant of neuroendocrine tumor ("carcinoid") described in several organs, but only recently observed in the appendix. In this study, we report the morphologic, immunohistochemical, and ultrastructural features of an incidentally discovered appendiceal clear cell/lipid-rich carcinoid in a 32-year-old man without any evidence of von Hippel-Lindau disease. Differential diagnosis with mimicking neoplastic and non-tumor lesions, epidemiology, and clinical behavior of this exceedingly rare variant of carcinoid of the appendix are also discussed.