Children with sickle cell anemia (SCA) often exhibit nutritional deficiencies and are at high risk of dying before the age of 5 years. Ensuring adequate nutrition is a critical part of health care for such children. This study aimed to investigate the association between nutritional status, nutrient intake, and food diversity in children with SCA. A descriptive cross-sectional study was conducted on 74 children with SCA, between 24 and 71 months of age. Anthropometric measurements, food and nutrients consumption were determined. The prevalence of low weight, stunting, and overweight/obesity were 16.2%, 35.1%, and 16.2%, respectively. Mean folic acid intake was low (49.05%±51.22%), whereas the intakes of protein (426.71%±171.93%), retinol (292.97%±403.88%), phosphorus (204.55%±151.35%), magnesium (233.02%±151.14%), iron (250.76%±165.81%), and zinc (243.21%±148.40%) were high. The dietary phosphorus/protein ratio was high for 31.1% of the children, and 44.6% of the children had low dietary diversity score. No correlation was found between food diversity, nutrient adequacy, and nutritional status. Despite the adequacy of the intake of most micronutrients, diet quality was inadequate, constituting mainly ultraprocessed foods. Knowing the food consumption pattern of these children enables a more resolute nutritional intervention.