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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.A literature review was conducted using MEDLINE, PubMed, CINAHL, ProQuest, and Google Scholar. The search resulted in 593 articles; 25 relevant articles were chosen.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.A paucity of research and case reports exists, limiting recommendations for optimal diagnosis, management, and treatment regimens.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.