A 76-year-old male patient presented with a nontender and firm left chin mass for 3 weeks. Ultrasonography revealed a 2.9 cm hypoechoic mass with minimal interior Doppler signal in the left submandibular gland. Contrast-enhanced computed tomography demonstrated a 2.9 cm homogeneous contrast-enhanced mass in the left submandibular gland; a left submandibular gland tumor was suspected. A total resection of the left submandibular gland tumor was performed. Immunohistochemical studies revealed strong positive staining for immunoglobulin (Ig) G4. A pathological diagnosis of IgG4- related sclerosing disease was made.