Paragangliomas of the head and neck area should be detected early and diagnosed in their complexity at specialized centers, treated and, in the presence of paraganglioma syndrome, cared for for life. The continuing education article focuses in an abridged form on the most important basics of a complex clinical picture and deals with both sporadic head and neck paragangliomas and hereditary paraganglioma syndromes. It contains concise recommendations regarding genetic testing, the diagnosis of multicentric tumors, the multidisciplinary treatment approach and the need for lifelong monitoring.