Here, we report the case of a patient with a slowly-progressing anterior mediastinal teratoma submitted to surgical resection. The anatomopathological examination of the sample revealed malignant degeneration to carcinoid tumor. Such evolution is very rare, and we have found only three related studies in the literature. We describe the clinicopathological features of the tumor and discuss the treatment administered. The evolution was satisfactory, and the patient was submitted to oncological treatment.