AbstractThe occurrence of diffuse large B-cell lymphoma (DLBCL) of the appendix presenting as acute appendicitis is rare, constituting only a minuscule portion (0.015%) of gastrointestinal lymphoma cases. We present a case of a 55-year-old woman that was admitted to the emergency department with symptoms of acute appendicitis and underwent an interval laparoscopic appendectomy with a right hemicolectomy and lymph node resection. Pathological examination revealed the presence of DLBCL in the appendix. The patient is undergoing chemotherapy.