In this cohort we identified a homozygous variant in the gene encoding GPR10 (NM_004248.3: c.665_670dup / p.R222_Q223dup) in a 5-year old girl with hyperphagia and weight gain from the age of 2.5 years, resulting in severe obesity (BMI SDS: 5.5). The gene discussed is PRLHR; the disease is Obesity.