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Background: There are few detailed consensus and guidelines on perioperative clinical characteristicsof liver transplantation (LT) in patients with methylmalonic acidemia (MMA). This retrospective studyinvestigated details of the clinical course and individualized treatment plan of the center with largestexperience in China.Methods: A total of 7 MMA patients undergoing LT in Beijing Friendship Hospital from June 2013 toDecember 2017 were enrolled in the study, whose clinical data (clinical characteristics, laboratory findings,chronological changes in urine MMA levels, treatment, etc.) during perioperative period were analyzedretrospectively. All the patients received strict postoperative management.Results: All the 7 cases were confirmed to have isolated MMA, among which, 3 cases received livingdonor liver transplantation (LDLT), 4 cases received deceased donor liver transplantation (DDLT). A wildfluctuate of metabolic condition was observed within the first few days after surgery and two weeks afterLT, the mean base excess of blood value (BE-B) restored to normal whereas plasma bicarbonate (HCO3-)was still below normal value even with intermittent sodium bicarbonate correction. It also showed markedreduction in propionylcaitine (C3) and C3/C2 level and the mean urine MMA by gas chromatographymassspectrometry (GC-MS) was reduced by 81.7％ (P72× higher than upper limitof normal. The metabolism-correcting medications were administered as before. The renal function ofone case with renal insufficiency before LT (serum creatinine rising) maintained stable by adjusting theimmunosuppressive regimen during the observation period. All patients survive to date.Conclusions: LT is an effective treatment to prevent metabolic crisis, but patients with MMA tend to bemetabolically fragile even after surgery. During perioperative period, close monitoring should be given foracidosis episodes so as to implement sodium bicarbonate correction. Metabolism-correcting medications arestill needed. Special immunosuppressive regimen is an effective way of maintaining renal function for thosewith kidney dysfunction.