Sjögren's syndrome is a chronic autoimmune disease, and Küttner tumor is a benign inflammatory condition. Both affect the salivary glands and are rarely associated. A 48-year-old Black woman complained of bilateral submandibular salivary gland enlargement, xerostomia, and xerophthalmia for approximately 7 months. Sialometry revealed severe hyposalivation. An ultrasound examination performed 4 months after the onset of symptoms showed a solid hypoechogenic nodule of 20 mm in the left submandibular salivary gland. An incisional biopsy demonstrated a chronic sclerosing sialadenitis (Küttner tumor) with only 10% positivity for IgG4+ plasma cells, ruling out the diagnosis of IgG4-related disease. Serologic exam for anti-SSA/Ro antibody was highly reactive (200.0 U/mL). Labial salivary glands were excised, and the histopathologic exam revealed a focal lymphocytic sialadenitis, confirming the diagnosis of Sjögren's syndrome. Prednisone (40 mg) and methotrexate (15 mg) were administered for 1 month, with symptom improvement and clinical reduction of the volume of the Küttner tumor. Sjögren's syndrome is a chronic autoimmune disease, and Küttner tumor is a benign inflammatory condition. Both affect the salivary glands and are rarely associated. A 48-year-old Black woman complained of bilateral submandibular salivary gland enlargement, xerostomia, and xerophthalmia for approximately 7 months. Sialometry revealed severe hyposalivation. An ultrasound examination performed 4 months after the onset of symptoms showed a solid hypoechogenic nodule of 20 mm in the left submandibular salivary gland. An incisional biopsy demonstrated a chronic sclerosing sialadenitis (Küttner tumor) with only 10% positivity for IgG4+ plasma cells, ruling out the diagnosis of IgG4-related disease. Serologic exam for anti-SSA/Ro antibody was highly reactive (200.0 U/mL). Labial salivary glands were excised, and the histopathologic exam revealed a focal lymphocytic sialadenitis, confirming the diagnosis of Sjögren's syndrome. Prednisone (40 mg) and methotrexate (15 mg) were administered for 1 month, with symptom improvement and clinical reduction of the volume of the Küttner tumor.