BACKGROUND Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is rare; to date, most reports have been single-case reports, and no multi-case reports or literature reviews are available. METHODS We conducted a retrospective analysis of 5 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma treated at our hospital between 2011 and 2016. All 5 patients had varying severities of leukopenia, anemia, thrombocytopenia, prolonged prothrombin time, and hypofibrinogenemia. METHODS All the patients underwent surgery: 2 patients had left hemihepatectomy; 1 had enucleation; 1 had a right hemihepatectomy; and 1 had a left trisectionectomy. RESULTS The 5 patients had an average operative time of 6.9 hours and an average blood loss of 3200 mL. One patient developed a biliary fistula (grade II) after the operation. There was no mortality among 5 patients. The white blood cell counts, hemoglobin, platelets, and prothrombin times of all 5 patients returned to normal after the operation. To date, a total of 11 cases of adult Kasabach-Merritt syndrome associated with giant liver hemangioma have been reported, of which 8 patients underwent surgery, and their platelets and coagulation returned to normal after the operation. CONCLUSIONS Adult Kasabach-Merritt syndrome associated with giant liver hemangioma is uncommon, and surgical treatment is risky. However, resection of the tumor corrected the abnormalities in hematological and coagulative systems.