Background: Accessory Hepatic Lobe (AHL) is a rare congenital anomaly that consists of the presence of a supernumerary lobe of hepatic parenchyma. The incidence of AHL is less than 1%, with only 27 cases having been reported in the paediatric population to date. Aim: To identify studies in the literature on accessory hepatic lobe and present our experience with this condition. Material and methods: We report the case of a patient who underwent surgery for intestinal occlusion and was found to have an accessory liver lobe with an embedded gallbladder. We reviewed published articles on accessory hepatic lobes accessed via PubMed, Google Scholar, Science Direct and Web of Science. Keywords included accessory hepatic lobe, ectopic liver tissue, ectopic liver lobe, accessory liver lobe. The search included articles published before August 2021 in English. Results: We identified 27 published cases of paediatric accessory hepatic lobe. Of these 27, 14 were female and 13 were male, and the age range was 1 day to 19 years. We report the case of a female patient with intestinal occlusion found to have an accessory hepatic lobe with an embedded gallbladder. Of the 28 published cases, including ours, 11 are associated with abdominal wall defects including Umbilical Hernia, Omphalocele, Cloacal Exstrophy and Beckwith Wiedemann syndrome. Conclusion: Accessory hepatic lobe is a rare entity usually detected incidentally. A total of 28 cases were reported, including our own. Paediatric surgeons should be aware of this condition in children with a history of abdominal wall defects.