Immunoglobulin G4–related disease (IgG4RD) is an immune-mediated condition characterized by lymphoplasmacytic infiltrates and fibrosis of affected organs. IgG4RD may affect many different organs either individually or together in a multiorgan condition and, thus, incorporates a wide range of fibroinflammatory phenotypes with shared pathologic features. Although IgG4RD most commonly occurs in late adulthood, it may affect children and adolescents. Only one case of IgG4RD presenting as isolated submandibular gland involvement has been reported in the pediatric population. Radiographic features of IgG4RD are often nonspecific making diagnosis challenging, but it is important for radiologists to be familiar with this diseased as its inclusion the differential for diffuse salivary enlargement may be the first step in making an accurate diagnosis. Here, we report a case of a child presenting with bilateral submandibular gland swelling to increase awareness of this condition in the pediatric population.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.