An understanding of the long-term outcomes of patients with advanced chronic kidney disease not treated with maintenance dialysis is needed to improve shared decision-making and care practices for this population. To evaluate survival, use of health care resources, changes in quality of life, and end-of-life care of patients with advanced kidney disease who forgo dialysis. MEDLINE, Embase (Excerpta Medica Database), and CINAHL (Cumulative Index of Nursing and Allied Health Literature) were searched from inception through December 3, 2021, for all English language longitudinal studies of adults in whom there was an explicit decision not to pursue maintenance dialysis. Two investigators independently reviewed all studies and selected those reporting survival, use of health care resources, changes in quality of life, or end-of-life care during follow-up. Studies of patients who initiated and then discontinued maintenance dialysis and patients in whom it was not clear that there was an explicit decision to forgo dialysis were excluded. One author abstracted all study data, of which 12% was independently adjudicated by a second author (<1% error rate). Forty-one cohort studies comprising 5102 patients (range, 11-812 patients) were included in this systematic review (5%-99% men; mean age range, 60-87 years). Substantial heterogeneity in study designs and measures used to report outcomes limited comparability across studies. Median survival of cohorts ranged from 1 to 41 months as measured from a baseline mean estimated glomerular filtration rate ranging from 7 to 19 mL/min/1.73 m2. Patients generally experienced 1 to 2 hospital admissions, 6 to 16 in-hospital days, 7 to 8 clinic visits, and 2 emergency department visits per person-year. During an observation period of 8 to 24 months, mental well-being improved, and physical well-being and overall quality of life were largely stable until late in the illness course. Among patients who died during follow-up, 20% to 76% had enrolled in hospice, 27% to 68% died in a hospital setting and 12% to 71% died at home; 57% to 76% were hospitalized, and 4% to 47% received an invasive procedure during the final month of life. Many patients who do not pursue dialysis survived several years and experienced sustained quality of life until late in the illness course. Nonetheless, use of acute care services was common and intensity of end-of-life care highly variable across cohorts. These findings suggest that consistent approaches to the study of conservative kidney management are needed to enhance the generalizability of findings and develop models of care that optimize outcomes among conservatively managed patients.