Background. Sickle cell disease patients are susceptible to the development of vitamin D deficiency. Vitamin D through binding to vitamin receptor (VDR) exerts its function and affects gene transcription in target tissues. Also, VDR variants could affect bone mineral density. Methods. In a case-control study 101 sickle cell disease patients including 61 SS, 39 S/β-thalassemia, and 1 SD along with 110 healthy individuals from Kurdistan of Iraq were studied. The lipid profile, vitamin D level, FokI, and TaqI variants of VDR and group-specific component (GC) were detected using the standard enzymatic method, the immunodiagnostic systems limited EIA kit and PCR-RFLP methods, respectively. Results. Around 93 and 82% SS and S/β thalassemia patients, respectively had vitamin D deficiency compared to 83% healthy individuals. Severe vitamin D deficiency (<10 ng/ml) was detected in 78.7% of SS patients. Plasma levels of total cholesterol, HDL-C, and LDL-C in SCD patients were significantly lower compared to controls. Vitamin D levels were negatively correlated to TG and positively correlated to total cholesterol and HDL-C. The frequencies of the C allele of FokI were 81.7 (p=0.003), 80.2 (p=0.034), and 84.6% (p=0.011) in all SCD, SS, and SS/βthal patients, respectively compared to 69.1% in controls. However, no significant difference was detected by comparing the frequencies of VDR TaqI and GC polymorphisms between SCD patients and controls.Conclusion. In the present study we found hypocholesterolemia, high prevalence of VDR FokI C allele, and low vitamin D level among children and adults with SCD patients from Kurdistan of Iraq.