Introduction: Primary gastrointestinal lymphoma is rare and appendix involvement is rarer. Most cases of appendiceal lymphoma present as clinical acute appendicitis and are generally diagnosed intra-operatory. Some computed tomography findings in preoperative evaluation may suggest appendix neoplasm rather than appendicitis, such as appendix diameter larger than 15 mm, abdominal lymphadenopathy and aneurysmal dilatation of the lumen.
Case Report: We report a case of a 26-year-old Brazilian man first diagnosed with acute appendicitis and submitted to videolaparoscopic approach with appendectomy and posterior chemotherapy after histopathological reveal of primary appendiceal lymphoma.
Conclusion: Surgical resection followed by chemotherapy remains as standard management choice when possible. Adequate investigation and mandatory histopathological analysis in appendectomies are fundamental for diagnosis and proper treatment.