INTRODUCTION: Carcinoma showing thymous-like elements (CASTLE) is a rare tumor with only a few cases described in the scientific literature. The aim of the present review is to analyze the data available on the therapeutic options employed in CASTLE tumors and to outline the best surgical management to adopt. MATERIALS AND METHODS: English-language articles published from 1985 through November 2016, and related to CASTLE cases were retrieved using the Pubmed database and specific key-words. RESULTS: Eighty seven cases included in 23 papers, published in the period under investigation, were analyzed in detail. The mean age of the patients was 50.2 years and the male to female ratio 1:1.2. There were performed 31 (35.6%) lobectomies, 29 (33.3%) total thyroidectomies, 15 (17.2%) subtotal thyroidectomies, 4 (4.6%) excisions, 3 (3.4%) partial thyroidectomies, 1 (1.1%) hemithyroidectomy, and 8 (9.2%) additional procedures including tracheal, pharyngeal, and esophageal resections. Lymph node dissection and radiotherapy were associated in 59 (67.8%) and 44 (50.6%) cases respectively. Among the patients with available data 62 (75.6%) were free of disease, 16 (19.5%) were alive with disease, 3 (3.7%) died for the disease, and 1 (1.2%) died for cerebrovascular complications at the time of follow-up. Globally 20 (24.4%) cases of local or distant recurrence were reported. Surgery and radiotherapy, alone or in combination were the treatments most frequently used for recurrences. CONCLUSIONS: CASTLE is a rare tumor which generally respond well to complete surgical resection and adjuvant radiotherapy. These treatments can be further employed for the recurrences, which occur in 24.4% of the cases, along with specific chemotherapy regimens and palliative procedures.