Inflammatory myofibroblastic tumours (IMFTs) are rare, distinct clinicopathological entities characterised by a dense inflammatory cell component amid myofibroblastic proliferation. Although the lung is the site commonly associated, there are reports of extra pulmonary involvement. IMFTs of the gastrointestinal tract are extremely rare and there have been only three confirmed cases of involvement of the appendix. These benign tumours have distinctive clinicopathological features that distinguish them from inflammatory fibroid polyps, gastrointestinal stromal tumours, smooth muscle tumours, sclerosing mesenteritis, lymphoma and other malignant tumours.^1–6 This letter highlights the clinical and immunohistological features of an IMFT of the appendix.

A 34-year-old man presented with progressively worsening colicky pain in the right lower abdomen for the past 3 days, associated with low-grade fever and two episodes of vomiting. Clinical examination showed a tender right lower quadrant with a vague mass. Investigations were unremarkable except for a raised total white cell count and an increased erythrocyte sedimentation rate (12 800 cells/mm³ and erythrocyte …