An 8-year-old girl was introduced to our department due to the presence of a left painless submandibular mass. The mass had been initially noticed at 7 years of age. Preoperative imaging showed the mass to have originated from the left submandibular gland. The mass was removed with a part of submandibular gland attached to it. The pathologic findings showed the mass to be pleomorphic adenoma without any malignant components. The postoperative clinical course was uneventful. During the 1-year follow-up period, no recurrence was noticed. In addition to the clinical report of our case, we reviewed the pertinent Japanese literature to clarify the clinical features of this disease in children.