Background and Aims Intrahepatic ectopic splenosis (ES) is relatively rare in clinical practice. In most patients, the intrahepatic lesions are accidentally found during physical examination, often misdiagnosed as benign or malignant liver tumors, and may be given inappropriate treatment. Therefore, this study was conducted to investigate the clinical characteristics of intrahepatic ES and its diagnosis and treatment methods.Methods The clinical data of 6 patients with intrahepatic ES treated in Shanghai Eastern Hepatobiliary Surgery Hospital from January 2015 to January 2022 were retrospectively analyzed and summarized, and the relevant literature review was performed.Results Of the 6 patients with intrahepatic ES, 4 cases were males, and 2 cases were females, aged 39-78 years, with a median age of 51 years; all cases had a history of splenectomy for traumatic splenic rupture and hemorrhage, and the time from splenectomy to initial diagnosis of intrahepatic ES was 20-33 years, with a median time of 27 years; 4 cases were pure intrahepatic ES, and 2 cases were multiple ES of the liver and diaphragm; one case was accompanied by discomfort of the right upper abdomen, and all the other 5 cases were found in physical examination; 5 cases were initially diagnosed as liver cancer, and 1 case was as initially diagnosed as angiomyolipoma by preoperative MRI or CT. All 6 patients underwent hepatectomy. Postoperative pathological examination confirmed that all the specimens were ectopic splenic tissue. The size of the intrahepatic splenic implant was 1.5-5.9 cm, with a median diameter of 3.2 cm. In one patient, the two lesions were ES and hepatocellular carcinoma, respectively, as determined by pathological examination. All 6 patients recovered uneventfully after the operation, and no new ES lesions were found during half a year follow-up period. The results of literature review showed that 90% of the ES occur in the abdominal cavity, which is frequently found in the serosa of the small intestine, the greater omentum, the parietal peritoneum, the mesentery, and the pelvic cavity, but rarely detected in the liver, the diaphragm, and other remote organs.Conclusion In individuals with a previous history of splenectomy for splenic trauma, the possibility of heterotopic ES should be considered after detecting intrahepatic lesions; Intrahepatic ES grows slowly, and most of them have no clinical symptoms. No special treatment is needed for those without clinical symptoms after a definite diagnosis.