Epidermolysis bullosa is an inherited disorder which is characteristically presented as skin blisters developing in response to minor injury. Junctional variety of Epidermolysis bullosa is associated with enamel hypoplasia. Amelogenesis imperfecta presents with abnormal formation of the enamel both in deciduous and permanent dentition. This is a case report of amelogenesis imperfecta with complete loss of enamel in a young female patient with epidermolysis bullosa.