Somatic malignancy arising from presacral or retroperitoneal primary teratoma is extremely rare. We report the case of a 37-year-old male patient with adenocarcinoma of respiratory type arising from primary presacral teratoma, but which first presented as anal fistula and rectal adenocarcinoma. The two tumors show the same morphology and immunophenotype (CK7-CK20+CDx2+). Malignant adenocarcinoma transformations from the normal respiratory epithelium are also found. To the best of our knowledge, this is the second case of respiratory type adenocarcinoma arising from primary presacral mature cystic teratoma.