Acromegaly is a rare chronic disease and associated with an increased risk of malignancy. The issue of the risk of thyroid cancer in these patients is a topic of debate, and the number of large case–control studies is very limited. Several studies indicated that a chronic excess insulin-like growth factor-1 stimulates the proliferation of various cell types and induces an antiapoptotic effect in thyroid follicular cells. In the literature, the risk of thyroid cancer was reported greater than five-fold. In this review, we will briefly summarize the studies available regarding thyroid cancer in patients with acromegaly and present three case reports.