Sarcopenia, commonly found in the elderly and characterized by the loss of skeletal muscle mass, decreased muscle strength, and reduced physical performance, draws attention because it often leads to frailty, an increased risk of falls and fractures, and higher morbidity and mortality. Patients with chronic kidney disease (CKD) usually suffer from malnutrition, physical inactivity, inflammation, metabolic acidosis, insulin resistance, and other hormonal changes, which are all aggravating factors of sarcopenia. Therefore, the prevention, early detection, and adequate management for sarcopenia in patients with CKD help to improve their quality of life, prevent various complications and disabilities, as well as to reduce the risk of major morbidities and death. We reviewed the diagnosis and prevalence of sarcopenia in CKD and discussed the risk factors, etiology, pathophysiology, clinical impacts, and present treatment strategies for sarcopenia in CKD. The applications of exercises along with nutritional interventions, correction of metabolic acidosis caused by CKD, anabolic hormones, appetite stimulants, and other agents in uremic sarcopenia were discussed. Early detection and adequate management help to improve muscle strength and mass and enhance physical performance, therefore improving the quality of life and reducing mortality in CKD patients with sarcopenia.