Pulmonary sequestration is a rare and usually asymptomatic congenital anomaly. Optimal management of this condition is still a subject of debate, including superiority of surgical resection or angiographic embolization of the aberrant arterial vessel. Presently described is rare case of a 51-year-old male who presented with hemoptysis related to pulmonary sequestration associated with acute right lower quadrant abdominal pain caused by perforated appendicitis.