Tailgut cysts (TGCs) are rare congenital lesions arising from remnants of the postanal gut, which normally involutes by the 8th wk of embryonic development (the 3-8 mm stage). They are usually found in the retrorectal or presacral space. They often present in middle aged women with perirectal symptoms. CEA producing adenocarcinoma from a TGC is extremely rare, but such cases have recently been reported in the literature. The diagnosis has to differentiate between carcinomas of the colorectum, hamartomas, lymphomas, teratomas, chordomas, abscess formation, dermoid cysts, epidermoid cysts and enteral cysts. An elevated level of CEA may point to malignancy, but other causes must be excluded. Complete surgical resection is the therapy of choice. We report here on a case of a tailgut cyst that occurred in a 72-year-old male suffering from pneumoconiosis, and this patients showed unusual findings such as an unusual location and an elevated serum CEA level.