A 28-year-old woman was admitted to our hospital because of recurrent hemoptysis during her menstrual period in the past three months. Chest CT images showed a ground-glass opacity in left lung S⁸ and a pulmonary arteriovenous malformation (PAVM) in left lung S⁶. Bronchoscopic examination performed during her menstruation and dilatation of submucosal vessels was observed in the lower trachea and bilateral main bronchus. A bronchoalveolar lavage fluid from the left B⁸ showed fresh bloody fluid containing hemosiderin-laden macrophages; therefore clinically a diagnosis of pulmonary endometriosis was suspected. Antiandrogen therapy using a gonadotropin-releasing hormone agonist was applied and improved her hemoptysis. We also performed a transcatheter coil embolization for the PAVM. After this treatment, her clinical state has been stable without recurrence for 5 years. We report a case of recurrent hemoptysis during each menstrual period with pulmonary arteriovenous malformation for the first time in Japan, which was treated with a minimal invasive technique.