The purpose of this article is to update the medical community on the current management of patients with appendiceal neoplasms. The authors discuss clinical evidence of medical and surgical treatment with emphasis on presentation, diagnosis, pathology, and surgical technique. Current available clinical evidence on the use of systemic chemotherapy is included. The authors describe in detail management of peritoneal carcinomatosis arising from tumors of the appendix with cytoreductive surgery and hyperthermic intraperitoneal therapy as standard of care.