• Most primary neoplasms of the appendix are detected incidentally on imaging or after pathologic diagnosis after an appendectomy.  • Primary neoplasms are found in 0.9–1.4% of appendectomy specimens.  • Primary neoplasms of the appendix are either epithelial, non-epithelial, and mixed lesions.  • At the time of diagnosis, 74% of appendiceal cancer cases have developed regional or distant metastases.  • CT or MRI should be used to evaluate a suspected appendiceal neoplasm.  • Appendiceal neoplasms are often not seen on colonoscopy but on occasion, they can be seen as protrusion of the appendiceal orifice.