Xanthogranulomatous inflammation is rare, and though it has been described in many organs, most commonly it occurs in the kidney and gall bladder. Xanthogranulomatous appendicitis is a very rare phenomenon. Clinical findings and imaging modalities are not sufficient for definite preoperative diagnosis. Clinical and radiological resemblances of these lesions to neoplastic processes often warrant excision. A 40-year-old female presented with pain in the right lower quadrant of the abdomen, loss of weight, nausea, and abdominal distension since 15 days. Ultrasonography and computed tomography of the abdomen and pelvis suggested neoplastic lesion. Surgical resection of the ileum, ileocecal junction with appendix, and part of transverse colon were done. Diagnosis of xanthogranulomatous appendicitis was made on histopathology examination.
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