Purpose: To present a case of twin-to-twin transfusion syndrome (TTTS), followed by discussion of the etiology, pathophysiology, prenatal diagnosis, management, complications, prognosis, and family considerations. Methods: A literature review was conducted using MEDLINE, PubMed, CINAHL, ProQuest, and Google Scholar. The search resulted in 593 articles; 25 relevant articles were chosen. Results: Approximately 1 of every 250 cases of monochorionic twins acquire TTTS. The progression of TTTS is unpredictable; nontreatment and early onset have been associated with >90 percent mortality of both twins. Neurodevelopmental impairment, preterm birth, renal hypoperfusion, growth restriction, cardiomyopathy, and hydrops fetalis have been associated with TTTS. Limitations: A paucity of research and case reports exists, limiting recommendations for optimal diagnosis, management, and treatment regimens. Implications for Practice: TTTS is unpredictable in progression; earlier identification of chorionicity, diagnosis of TTTS, and improvement of treatment and management have the potential to improve the mortality and morbidity outcomes associated with TTTS.