Introduction Collision tumors in the thyroid gland are exceedingly uncommon, comprising approximately 1.0% of all thyroid malignancies. This study aims to systematically review the presentation and management of collision tumors of the thyroid gland. Methods A systematic review of published studies on thyroid collision tumors was conducted. All studies concerning thyroid collision tumors meeting the following criteria were included: 1) Confirmation of collision tumors via diagnostic methods, surgical exploration, or histopathological examination. 2) Presentation of case details within the study. 3) Tumors located precisely in the thyroid gland. Results In total, 57 studies were compatible with the inclusion criteria. Most cases were female 87 (71.31%), and the remaining 35 (28.69%) were male. The patients' ages were between 12 and 88 years old, with a mean of 49.87 ± 14.48 years. The most commonly presented symptom was neck swelling (45.08%). The most prevalent surgical procedure observed was total thyroidectomy combined with lymph node dissection, performed in 40 cases (32.78%). The predominant histopathological findings consisted of the simultaneous presence of papillary thyroid carcinoma and medullary thyroid carcinoma, identified in 51 cases (41.80%). The recurrence rate was observed in only 10 cases (8.20%). Conclusion Thyroid collision tumors may primarily affect females, with the most frequent collisions being papillary and medullary thyroid carcinoma. Total thyroidectomy with lymph node dissection is the prevalent management option, and the recurrence rate can be lower than 10%.
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