Sarcopenia, a pathologic deficiency of muscle mass and function, has emerged as an important secondary feature of many chronic disease states. For adults with end stage liver disease, there are multiple mechanisms which contribute to sarcopenia and its presence has proven to be an important predictor of morbidity and mortality. In children, there are only a limited number of reports which investigate the role of sarcopenia in liver disease. These studies, which are discussed and summarized in this review, report small, single-center analyses with dissimilar study cohorts and varying clinical definitions. Still, children meeting the study entry criteria have sarcopenia with a reported prevalence of 24–70%. When assessed, sarcopenia appears to be associated with more severe disease but is independent of the Pediatric End-Stage Liver Disease (PELD) score and does not correlate with age, gender, or traditional anthropometric measures such as weight, height, weight-for-height, or body mass index (BMI). While individual studies may identify sarcopenia as a statistically significant risk factor for certain post-transplant outcomes such as longer ICU stay, longer duration of intubation, repeat operation, development of serious infection, longer hospital stay, death, or long-term growth failure, such associations are not consistently replicated across studies. Finally, although various methods of muscle mass quantification are utilized, the most reported is the total psoas muscle surface area (tPMSA) on computed tomography. This method, along with others such as skeletal muscle area and skeletal muscle index, have had normative values recently defined and these collective efforts should enable researchers a common basis of comparison when delineating sarcopenia, and its impact, across various study populations in future investigations – including in children with liver disease.