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Situs inversus totalis is a condition in which both the abdominal and thoracic organs are located as perfect minor images of their normal position. This is not a common condition, but at the same time, an important one since it is associated with various important pathologic conditions as are male infertility and Kartagener's syndrome. At the same time, this condition has very important consequences if it starts with acute manifestations. A male patient goes to the emergency room with a history of abdominal pain. Physical examination reveals rebound tenderness in the left lower quadrant. Laboratory and image studies confirm the diagnosis of situs inversus totalis and acute appendicitis. Recognizing situs inversus and it's medical implications become great important to primary care physicians.