Chronic kidney disease (CKD) is a significant complication in patients with sickle cell disease (SCD), leading to increased mortality. This study aims to investigate the burden of CKD in Medicaid-enrolled adults with SCD in California, examine differences in disease burden between male and female individuals, and assess mortality rates and access to specialized care. This retrospective cohort study used the California Sickle Cell Data Collection program to identify and monitor individuals with SCD. Medicaid claims, vital records, emergency department, and hospitalization data from 2011 to 2020 were analyzed. CKD prevalence was assessed based on ICD (International Classification of Diseases) codes, and mortality rates were calculated. Access to specialized care was examined through outpatient encounter rates with hematologists and nephrologists. Among the 2345 adults with SCD, 24.4% (n=572) met the case definition for CKD. The SCD-CKD group was older at the beginning of this study (average age 44, SD 14 vs 34, SD 12.6 years) than the group without CKD. CKD prevalence increased with age, revealing significant disparities by sex. While the youngest (18-29 years) and oldest (>65 years) groups showed similar CKD prevalences between sexes (female: 12/111, 10.8% and male: 12/101, 11.9%; female: 74/147, 50.3% and male: 34/66, 51.5%, respectively), male individuals in the aged 30-59 years bracket exhibited significantly higher rates than female individuals (30-39 years: 49/294, 16.7%, P=.01; 40-49 years: 52/182, 28.6%, P=.02; and 50-59 years: 76/157,48.4%, P<.001). During this study, of the 2345 adults, 435 (18.5%) deaths occurred, predominantly within the SCD-CKD cohort (226/435, 39.5%). The median age at death was 53 (IQR 61-44) years for the SCD-CKD group compared to 43 (IQR 33-56) years for the SCD group, with male individuals in the SCD-CKD group showing significantly higher mortality rates (111/242, 45.9%; P=.009) than female individuals (115/330, 34.9%). Access to specialist care was notably limited: approximately half (281/572, 49.1%) of the SCD-CKD cohort had no hematologist visits, and 61.9% (354/572) did not see a nephrologist during this study's period. This study provides robust estimates of CKD prevalence and mortality among Medicaid-enrolled adults with SCD in California. The findings highlight the need for improved access to specialized care for this population and increased awareness of the high mortality risk and progression associated with CKD.