Head and neck paragangliomas (HNPGs) are rare tumours of neural crest origin that are benign in the majority of cases. In this article HNPGs are outlined with emphasis on genetic predisposition, diagnostics and counselling. The literature on HNPGs identified using PubMed is reviewed. Several susceptibility genes have been identified and hereditary mutations are detectable in 30% of the patients with HNPGs. Genetic analyses, guided by the family history and clinical findings, should be offered to all patients with HNPGs and at risk relatives.