Objective: to investigate situation, clinical manifestations of congenittal epidermolysis bullosa (EB) at the NHDV. Material and method: the cross-sectional study (retrospective and prospective) basing on data of all patients diagnosed as EB at the NHDV from 01/2007 to 07/2011. Results: during 4, 5 years, there were 100 patients with EB, accounting for 6.62 percent of bullous skin disease, 0.02 percent of patients visit at the NHDV. Average age was 1.67 + or - 2.56. Twenty five percent of patients was under 1 month. There was no difference between male and female with the rate: 1.1. Seventy four of patients came from rural area. Blister met in 100 percent of case in which trauma - prone surfaces account for 85.7 percent. The colour of fluid was mainly clear (45.7 percent). Other symptoms included mucous membrane lesions (48.7 percent), nail lesions (14.3 percent). There were 45.7 percent of patients with fever and leukocytosis above 10g G/l. Conclusion: EB account for 6.62 percent of bullous skin diseases. The disease was mainly occurred on children. The common symptom was blister at trauma - prone area. The mucous membrane lesion was occurred in 48.7 percent patients.