As noticed by our group [11] congenital profound HL in the patient is not a consequence of TMPRSS3/GJB2 digenic inheritance but results from compound heterozygous c.208delC (p.His70Thrfs*19) and c.579dupA (p.Cys194Metfs*17) TMPRSS3 pathogenic variants. The gene discussed is GJB2; the disease is Hodgkins lymphoma.