Objectives Silent sinus syndrome (SSS) is a rare condition involving idiopathic maxillary sinus underdevelopment, orbital floor resorption, enophthalmos, and hypoglobus. SSS is more commonly described in adults, with only very limited literature in the pediatric population. Given the rarity of the condition, there is a lack of consensus regarding proper diagnostic criteria and treatment strategies. We present a case of pediatric SSS and a review of other cases in the literature. Methods Case report and systematic review. Results A 12-year-old female patient was referred for incidental MRI findings suggestive of SSS. Examination revealed minimal enophthalmos with some hypoglobus. A CT scan of the sinuses confirmed the diagnosis of SSS, and the patient underwent a right sided endoscopic modified medial maxillectomy/mega-antrostomy with image guidance given the significant maxillary hypoplasia. A literature review of CINAHL, PubMed, and Scopus identified 15 studies that referenced pediatric SSS cases. Conclusions This article reports a case of pediatric SSS and compares the presenting symptoms, diagnostic criteria, and surgical intervention to other cases of pediatric SSS. As pediatric SSS is a very rare disease, this case offers a possible treatment strategy for a severely atelectatic maxillary sinus.