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Isolated tuberculosis (TB) of the appendix is a rare occurrence. Its reported incidence is only 0.1-0.6%. The diagnosis is usually made only after histopathological examination of the resected specimen. The present case is of a 14-year-old male child who presented with signs and symptoms of acute appendicitis and was operated on. He was diagnosed with TB of the appendix on histopathological examination with no detectable focus elsewhere in the body. We report this case because of its rarity. The current article also emphasizes the importance of histopathological examination of all appendicectomy specimens.