Patients with sickle cell disease have vitamin D deficiency and poor bone health which makes them prone to have an increased risk of fractures and osteoporosis in adulthood. We performed a prospective, cross-sectional study in children diagnosed with sickle cell disease living in Madrid, Spain. The purpose of this study was to evaluate the status of vitamin D of these children. Patients 0-16 years old were enrolled between 2008 and 2011. We studied demographics, calcium metabolism, and bone health, especially by measuring levels of 25-hydroxyvitamin D (25(OH)D), during different seasons of the year, and bone densitometry (beyond 4 years of age). Seventy-eight children were included in the study. Mean age was 4.8 ± 4.3 years, and mean serum 25(OH)D level was 21.50 ± 13.14 ng/ml, with no differences in 25(OH)D levels within different seasons. Fifty-six percent of children had levels of 25(OH) vitamin D of <20 ng/ml, whereas 79 and 18 % of them had levels of <30 and <11 ng/ml, respectively. Secondary hyperparathyroidism was observed in 25 % of children. Densitometry was performed in 33 children, and an abnormal z-score was seen in 15.2 % of them with no correlation with levels of 25(OH)D. Conclusions: Vitamin D deficiency is highly prevalent in children with sickle cell disease, who are residing in Madrid, Spain, and it is detected at a young age. We propose that early intervention may increase the possibility of an adequate bone density later in life.