Appendiceal primary signet ring cell carcinoma is an extremely rare neoplasm considered to be more aggressive than other appendiceal tumour. Primary appendiceal carcinoma is diagnosed in only 0.9%–1.4% of appendectomy specimens and signet-ring cell carcinoma (SRCC) of vermiform appendix is accounting 0.43% of all appendiceal malignancies. Patients of SRCC usually present clinically as acute appendicitis. Preoperative imaging detection of appendiceal adenocarcinoma have limited benefit due it has minimal change like appendicitis or wall thickening without any obvious growth. We reported an extremely rare case of primary signet ring cell carcinoma of the vermiform appendix in a 55 year old man. Patient had abdominal pain, anorexia and nausea. He underwent appendectomy for appendicitis and histopathologically diagnosed as appendiceal signet ring cell carcinoma with lymph node metastasis.