Recently, a new subtype of HCA was described, which represents ca. 4% of HCAs, which are associated with obesity and bleeding risk, are defined by the activation of the sonic hedgehog signaling pathway, and are due to the fusion of the inhibin subunit beta E and glioma-associated oncogene 1 (GLI1) genes [9]. The gene discussed is GLI1; the disease is obesity due to melanocortin 4 receptor deficiency.