Dentigerous cyst (DC) in the maxillary sinus is extremely rare. This study evaluated the clinical features, etiologic factors, imaging, treatment, and signs and symptoms of this type of DC. Clinical symptoms, radiographic findings, surgical methods, and histopathologic findings of 4 patients with DCs in the maxillary sinus were evaluated. In addition, we reviewed the literature reports of DCs in the maxillary sinus over the past 40 years. Our 4 patients with DC in the maxillary sinus showed the following results: (1) all the patients were males at first 3 decades of age, (2) swelling and/or yellow-green pus discharges from pharyngeal cavity were common symptoms, (3) the involved teeth in the DC were the maxillary third molar teeth and supernumerary teeth, and (4) Caldwell-Luc approach was performed in these 4 patients. The patients with DC in the maxillary sinus should be evaluated thoroughly by extraoral and intraoral examinations, proper diagnostic imaging procedures, and pathologic examination to avoid misdiagnosis of maxillary sinusitis. The standard treatment for DC in the maxillary sinus is often removed by Caldwell-Luc approach. Furthermore, marsupialization or functional endoscopic sinus surgery may be performed because of the size, location of the cysts, or the age of the patients.