Introduction Teratoma is a rare tumor that is derived from germ cells. This tumor is composed of one or more than one types of tissue, such as sebaceous glands, hair, and teeth. Teratoma is commonly benign and presents in gonadal organs such as the testicles and ovaries, mostly. But, this type of tumor can be seen in other organs especially in the thoracic cavity (1, 2).Thoracic teratoma usually occurs in the mediastinum. In very rare cases, teratoma occurs in the lungs (3). Although pulmonary teratoma is very rare it was observed that this type of teratoma involves the upper lobes. The causes of the involvement are not defined until now (4).Pulmonary teratoma may be presented with cough, chest pain, or hemoptysis (3, 4). Based on our knowledge, all studies about intrapulmonary teratoma are case reports. In this study, we presented a 57-year-old man who was a known case of hypophosphataemic rickets for 16 years and was finally diagnosed with pulmonary teratoma.