Background: Necrosis of the falciform and round ligaments is extremely rare, thus making the diagnosis challenging. It is often misdiagnosed as gallbladder pathology due to the presenting symptoms. Due to the rarity of this pathology, there is limited literature available. Case presentation: A 53-year-old white man presented to our hospital with signs and symptoms of gallbladder pain but turned out to have the rare entity of necrosis of the falciform and round ligaments. An extensive review of the world literature was performed using PubMed. Manual cross-referencing of reference lists was performed to obtain all available articles. The personal operative log of the senior author was also searched to reveal one additional case. Statistical analysis was descriptive only, given the small number of reported cases. Thirty-nine articles were found, among which forty-three case were identified, and one additional case was extracted from the operative log of the senior author. Unlike previous reports, we found that isolated inflammation and necrosis of the ligaments occurs at nearly the same frequency in both men and women, not predominantly in women as previously reported in smaller series. The mean age at presentation was 59.5 years old, and cases were typically initially diagnosed as gallbladder pathology, most commonly acute cholecystitis. Computed tomography more frequently than ultrasound revealed the falciform and round-ligament pathology. Conclusions: Isolated falciform and round-ligament inflammation and necrosis is a rare condition that is difficult to diagnose because it can present mimicking a wide variety of intra-abdominal pathologies, particularly gallbladder pathologies. It is often best treated by laparoscopic resection. Unlike prior reports, our review of the literature, which is the largest that we know of to date, shows that males and females are equally affected. Greater awareness of this entity will aid in future diagnosis.