Marine-Lenhart syndrome (MLS) is an uncommon cause of primary hyperthyroidism, which can occur in the context of diffuse goiter due to Graves disease (GD) or autonomic nodular disease (Plummer disease (PD)). The coexistence of these two conditions is the hallmark of the MLS. Patients with MLS have a lower remission rate with oral antithyroid drugs, requiring definitive management therapies with radioactive iodine or surgery. We present the case of a 48-year-old female with a history of primary autoimmune hyperthyroidism (GD) since 2016, with biochemical control of hyperthyroidism with methimazole but without the possibility of stopping treatment. The scintigraphic uptake pattern showed heterogeneous uptake of the thyroid parenchyma with three hyper-uptake nodules without inhibition of the rest of the thyroid tissue, findings of an MLS condition with the indication for definitive therapy, for which he was referred to nuclear medicine for the administration of radioactive iodine.