Aim: We aim to report a novel association of unilateral Graves’ disease with papillary thyroid carcinoma affecting the same lobe. Background: Graves’ disease is characterized by diffuse enlargement of the thyroid gland. Unilateral Graves’ disease is a rare clinical entity characterized by enlargement and hyperfunctioning of a single affected thyroid lobe. Case description: A 29-year-old female presented with thyrotoxic symptoms for the last 1 year. The patient underwent detailed clinical, hormonal, and imaging studies, which confirmed the presence of unilateral Graves’ disease. During this evaluation, she was also found to harbor papillary thyroid carcinoma in the same affected lobe. The patient underwent total thyroidectomy, followed by radioactive iodine ablation. Histopathological examination of excised tissue confirmed the presence of papillary thyroid carcinoma. Conclusion: A diffuse goiter is one of the classical and well-characterized manifestations of Graves’ disease. Unilateral Graves’ disease is a rare variant of Graves’ disease characterized by unilobar involvement. The exact underlying mechanism of unilateral lobar involvement is unknown. Clinical significance: The association of papillary thyroid cancer and unilateral Graves’ disease is unique and has not been described earlier. Due to its rarity, the diagnosis might be easily missed or misdiagnosed. We have also reviewed and summarized the relevant cases of unilateral Graves’ disease reported earlier in the literature.