IgG4-related disease (IgG4-RD) is an immune-mediated disease condition that can affect almost any organ, including the head and neck. It is a chronic, systemic inflammation of unknown etiology. Tumor formation is the most common clinical symptom. Any tumor in the head and neck area is always a concern for an otorhinolaryngologist, head and neck surgeon, that we are dealing with malignant neoplastic growth. Ultrasound imaging, computer tomography, magnetic resonance imaging and even fine needle aspiration biopsy usually do not exclude neoplastic hyperplasia. Only open biopsy or excision biopsy followed by histopathological examination suggest the diagnosis of IgG4-RD, which requires further diagnosis, mainly serological. The authors present the most common IgG4-RD in the head and neck area, diagnostic criteria and their differentiation from apparently similar diseases.