Gastrointestinal tuberculosis is a common form of extra pulmonary tuberculosis. Tuberculosis of the appendix is a rare variant of gastrointestinal tuberculosis even in countries where tuberculosis is endemic like India; however, it should be included as one of the differential diagnosis of appendicular swelling as its clinical presentation mimicking various other conditions e.g. acute on chronic appendicitis, appendicular malignancy or abscess. The majority of the previously reported cases of tubercular appendicitis had a primary focus elsewhere in the body. Here we are describing a case of isolated tuberculosis of the appendix, who underwent laparotomy with the suspicion of appendicitis. The diagnosis was confirmed by histopathological examination of the resected specimen. No primary focus was found in elsewhere in the body. He was treated with four drug anti-TB regimen (2HERZ+4HR) and was asymptomatic in full course of therapy and later also till six months of follow up. Apart from rarity due to primary involvement, this case report highlights the rare clinical presentation and significance of histopathology in the diagnosis of this uncommon entity. Keywords: Primary tubercular appendicitis, Histopathology, Anti-TB regimen
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