A cross-sectional clinical assessment (body mass index, fat mass index, fat-free mass index, waist-to-height ratio, and blood pressure) and metabolic study (triglycerides, total cholesterol, HDL-C, LDL-C, glucose, insulin, HOMA-IR, leptin, calcium, phosphorous, calcidiol, and PTH) were carried out in 236 adolescents diagnosed with severe obesity (BMI z-score > 3.0, 99th percentile), aged 10.2–15.8 years. The gene discussed is INS; the disease is obesity due to melanocortin 4 receptor deficiency.