Introduction. Domino cross-auxiliary liver transplantation represents an innovative procedure for the treatment of selective noncirrhotic metabolic liver diseases. Methods. The treatment strategies and experiences in domino cross-auxiliary liver transplantations, including the world's first case in 2013, are reviewed. Results. Six patients with noncirrhotic metabolic liver diseases receiving domino cross-auxiliary liver transplantation included the following: familial amyloidosis with multiple neuropathy (case 1), ornithine transcarbamylase deficiency (cases 3, 5, and 6), and Wilson's disease (cases 2 and 4). Five patients achieved a favorable postoperative survival outcome and quality of life, whereas case 2 died of multiple organ failure at 3 months post liver transplant (LT). Case 1 experienced an imbalance in portal vein blood perfusion between the 2 domino livers at 6 months after LT but improved after interventional radiology treatment. Cases 3 and 4 showed domino grafts associated with hypercholesterolemia after LT, but total cholesterol levels decreased to normal ranges after dietary adjustment. Case 5 showed an effortless recovery after surgery with no complications during the follow-up period. Case 6 experienced an occult domino liver graft rejection, which resulted in graft dysfunction and eventual recurrence of the primary metabolic liver disease (ornithine transcarbamylase deficiency). A liver retransplantation may be required for this patient. Conclusions. Domino cross-auxiliary liver transplantation is an innovative and effective treatment for metabolic liver diseases in the patients who are strictly selected on the basis of pathophysiological and genetic criteria. Special attention to rejection monitoring and imbalance regeneration are required with this procedure.