Marine-Lenhart syndrome (MLS), a rare form of hyperthyroidism, is the coexistence of Graves’ disease (GD) and autonomously functioning thyroid nodule(s). Herein, we report a case of recurrent goiter presented with MLS. A 52-year-old man presented at our department with recurrent goiter, exophthalmia, and symptoms of hyperthyroidism. In addition to clinical signs and thyroid eye disease, suppressed thyroid-stimulating hormone (TSH) and high free thyroxine (FT4) and autoantibody levels lead to the diagnosis of GD. Thyroid ultrasound and nuclear scan showed the presence of a large, solid, and functioning “hot” nodule in the right lobe. Thus, in recurrent goiter cases, the diagnosis was MLS, wherein autoimmune hyperthyroidism was associated with the functioning nodule. Following medical control with methimazole, the patient underwent total excision of recurrent goiter. Levothyroxine (LT4) therapy was prescribed to maintain normal serum hormone levels. At follow-up, the gradual decrease in serum levels of autoantibody was detected. This patient is a very rare example of MLS that occurs in recurrent goiter case. Clinical signs, serum hormone and autoantibody levels, thyroid ultrasound, and nuclear scan establish the correct diagnosis of this specific and rare disorder. Thyroid surgery and total removal of glandular tissue provides definitive control of hyperthyroidism and obviates autoimmune reaction.