Objective: Studies report an increased prevalence of thyroid tumors among patients with acromegaly. Acromegaly often has a subtle presentation and is likely underdiagnosed. This report highlights the development of recurrent thyroid neoplasia in a patient with undiagnosed acromegaly and raises awareness of thyroid malignancy in patients with acromegaly.Case report: Mrs. R is a 47-year-old woman who presented with a recurrent goiter following two partial thyroidectomies, then was diagnosed with acromegaly and subsequently papillary thyroid cancer.Methods: A review of the English-language literature on the PubMed database of acromegaly and thyroid cancer was performed. DNA from the tumor and adjacent benign thyroid tissue was tested by polymerase chain reaction (PCR)/direct sequencing of genomic DNA for genetic abnormalities. Results:The relationship between the Growth Hormone (GH)/Insulin-like Growth Factor 1 (IGF-1) axis and the thyroid hyperplasia and neoplasia was detailed. The increased risk of malignancy occurs under the influence of specific goitrogens namely, prolonged GH/IGF-1 exposure. Conclusion:The reported link between acromegaly and thyroid tumors should raise suspicion of malignancy in acromegalic patients with thyroid nodules. Additionally, an increased suspicion of acromegaly should be entertained in patients with recurrent thyroid nodular hyperplasia following partial thyroidectomy.