INTRODUCTION AND OBJECTIVE: Osteosarcoma is a rare, malignant, primary bone tumor and usually affects long bones. There are two peaks in the incidence of this pathology, the first being in childhood and youth and the second, between the fifth and sixth decade of life; in childhood, the incidence is higher in females, and after 10 years, males are usually the most affected. It can be classified according to The Musculoskeletal Tumor Society (MSTS), which concisely classifies the prognostic factors; histologically, according to the dominant element present in the tumor; and it can also be classified using the Huvos Grade which categorizes the response to chemotherapy. It is already known that vascular endothelial growth factor (VEGF) is one of the major proangiogenic factors, and polymorphisms in the VEGF gene and its receptors (KDR) may influence tumor development. Thus, the aim of this study was to make an epidemiological, clinical and genetic profile (polymorphisms in VEGF-KDR genes) of patients with osteosarcoma. MATERIAL AND METHOD: The Human Research Ethics Committee of INTO approved this case study (17373613.8.0000.5273). Forty-eight patients diagnosed with osteosarcoma were recruited and analyzed as for age, sex, body-mass index (BMI), height, type of bone affected, metastasis, recurrence, histological classification, Huvos grade, MSTS classification and frequency of the VEGF-KDR polymorphisms. A genotyping of VEGF and KDR polymorphisms was performed using the TaqMan system. RESULTS AND CONCLUSION: The age of the cases was 20.04±10.14 years, either most frequent (58%) in the age group from 15 to 29 years old. Twenty-seven (56%) of the cases were female and the anthropometric characteristics such as BMI and height were 21.61±4.32 kg/m2 and 1.63±0.14 cm, respectively. Regarding the clinical features of the study population, 44 (92%) showed tumor location in long bones (67% femur, 17% tibia, 4% fibula and 4% humerus), 7 (15%) have metastasis, 35 (73%) have absence of recurrence, 15 (31%) presented MSTS classification as stage II B and histological classification as osteoblastic or chondroblastic (15% and 6%, respectively). In addition, among 23 cases who underwent chemotherapy were predominate the Huvos Grades III and I (27% and 6%, respectively). Variant alleles frequencies of the VEGF (1154G>A, 2578C>A, 460G>C, 405G>C, 936C>T) polymorphisms were 17.5%, 42.5%, 45%, 37.5% and 12.5%, respectively. While the variant alleles frequencies of the KDR 604T>C, 1192 C>T, 1719T>A polymorphisms were 52.5%, 25% and 15.8%, respectively. It is becoming increasingly important to derive data from different populations to build a database which can then be used in future investigations to a better understanding of the genetic, epidemiological, clinical and environmental factors affecting risk to development osteosarcoma, beyond to promote an individualized therapy, contributing to the reduction of recurrences and metastases.