Pulmonary sequestration is a type of bronchopulmonary malformation defined as an isolated portion of lung tissue with systemic arterial supply and no bronchial communication. Carbohydrate antigen 19-9 (CA19-9) has been used for diagnosis and follow-up of gastrointestinal tumors. The current study presents a rare case of intralobar pulmonary sequestration associated with the marked elevation of CA19-9. A 39-year-old female patient was admitted to our hospital due to acute liver injury with marked elevation of serum CA19-9 (3,051.1 μmol/mL), and was then diagnosed with intralobar pulmonary sequestration after examination and surgery. After the pulmonary resection, the serum CA19-9 levels decreased to normal ranges. We briefly reviewed the literature on elevated serum CA19-9 levels and pulmonary sequestration since 1988. We found that serum CA19-9 levels are increased not only in patients with digestive tract cancers but also in those with nonmalignant diseases such as pulmonary sequestration.