Background: Mucosa–associated lymphoid tissue (MALT) lymphomas comprise a group of indolent B–cell non–Hodgkin lymphomas, which have a low incidence in clinical practice. Thrombocytopenia generally does not develop in maltoma unless the tumour has infiltrated the bone marrow. Intussusception due to appendicular maltoma is extremely rare. Thrombocytopenia resulting from immunologic causes in patients with gastrointestinal(GI) maltoma have been rarely reported in the literature. Case report: 53 year old female presented with complaints of abdominal pain for 1 month along with altered bowel habits, fever, malena and decreased appetite. Abdomen examination showed tenderness in right iliac fossa. Ultrasound and CECT abdomen showed features suggestive of ileo colic intussusception. Exploratory laparotomy was done and it showed appendiculo–caecal intussusception with a hard tumour mass at the base of appendix and hence a radical right hemicolectomy was done. Biopsy report of the resected specimen showed high grade maltoma involving the base of appendix. Conclusion: The diagnosis of appendiceal maltoma is difficult in most of the cases. This case report is a very unique presentation of maltoma which adds richness to the limited amount of resources available.