Mirizzi Syndrome is a rare and challenging clinical entity to manage. However, recent advances in technology have provided surgeons with new options for more effective diagnosis and treatment of this condition. This paper reviews these new diagnostic modalities and treatment approaches for the management of Mirizzi Syndrome.An online search language was performed using PubMed and Web of Science for literature published in English between 2012 and 2017 using the search terms “Mirizzi Syndrome” and “Mirizzi.” In total, 16 case series and 11 case reports were identified and analyzed.The most frequently used diagnostic modalities were ultrasound, computed tomography (CT); magnetic resonance cholangiopancreaticography (MRCP); endoscopic retrograde cholangiopancreaticography (ERCP). A combination of ≥2 diagnostic modalities was frequently used to detect Mirizzi Syndrome. Literature shows that the specific type of Mirizzi Syndrome determined the type of treatment chosen. Open surgery was the preferred option, although there are documented cases of the use of minimally-invasive techniques, even in advanced cases. Laparoscopic, endoscopic or robot-assisted surgery, used individually or in combination with lithotripsy, were all associated with a favorable outcome.As yet, there are no internationally-accepted guidelines for the management of Mirizzi Syndrome. Laparotomy is the preferred surgical technique of choice, although an increasing number of surgeons are beginning to opt for minimally-invasive techniques. The number of papers in the existing literature describing diagnostic and treatment procedures is relatively small at present, thus making it difficult to reasonably propose an evidence-based standard of care for Mirizzi Syndrome.