Maxillary sinus hypoplasia (MSH) is a rare condition characterized by underdevelopment or decrease in the volume of the maxillary sinus. It is thought that MSH may be embryological in origin but acquired etiologies have also been discussed in the literature. MSH is usually detected upon radiographic examination of the maxillofacial area. Three types have been described based on variations in sinus anatomy. A case of bilateral maxillary sinus hypoplasia with concurrent hemimandibular hypertrophy is presented, and pertinent clinical and cone beam computed tomography findings are reported.