Transvenous pacing has become widespread in the pediatric population, but related pacemaker lead infection in young patients has rarely been reported. To determine prevalence and optimal management of pacemaker lead infection in children and young adults, the authors reviewed their pacing database including 4476 patients who previously had pacemaker implantations from 1975 to 2001. A pacemaker was implanted in 304 patients under the age of 40. Of these patients 217 of them had congenital heart disease: 108 with structural defect, 109 without (mainly complete AV blocks). Among patients with congenital heart disease, 12 developed a pacemaker lead infection (5.5%, 6 patients with structural defect, 6 without). This incidence was significantly higher than in patients < 40 years at first implantation without congenital heart disease (2.3%) and in > 40-year-old patients(1.2%, P < 0.001). However, the number of reinterventions at the pulse generator site was higher in patients having had their first implantation before the age of 40. In patients with structural cardiac defect: two died after surgical lead extraction and one died before the scheduled lead extraction. The three remaining patients had successful surgical (n = 1) or percutaneous (n = 2) lead extractions. In patients without structural cardiac defect successful percutaneous extraction (5/6) or surgical extraction (1/6 with vegetation > 25 mm) was performed. One patient with percutaneous extraction developed chronic cor pulmonale during follow-up. One infection recurred in one patient with structural cardiac defect although complete removal of the pacing material had been performed. The prevalence of pacemaker lead infection is higher in younger patients, perhaps in part due to a higher number of procedures at the pacemaker site than in the general population of patients with a pacemaker. Patients with structural cardiac defect who underwent surgical lead removal were at high risk for death. Patients with percutaneous lead extraction may develop cor pulmonale.