Hyperhomocysteinemia, resulting from a cystathionine beta synthase (CBS) deficiency, is an autosomal recessive disease associated with high levels of homocysteine. Such patients can present with severe mental retardation, ectopia lentis and osteoporosis and thromboembolic disease. To the best of our knowledge, only two cases of liver transplantation for CBS deficiency have been published. Here, we report a case of an 8-year-old male with a CBS deficiency that underwent living donor liver transplantation. The postoperative course was uneventful and homocysteine levels remained normal. The liver of this CBS deficiency patient was then successfully used in domino transplantation. The domino liver transplantation recipient was a 41-year-old male diagnosed with acute liver failure following hemi-liver resection due to cholangiocarcinoma. The domino recipient developed acquired hyperhomocysteinemia, which was controlled with a special regimen of medications. No complications relative to CBS deficiency were observed up to 11 months post-transplant. At this time, the patient expired as a result of cholangiocarcinoma recurrence. In conclusion, our data suggest that liver transplantation for CBS deficiency can be effective, safe and beneficial. It is possible to be both safe and beneficial to use a CBS deficiency patient as a domino donor for salvage liver transplantation in a selective category of recipients.