Background: Pyloric atresia is a rare but serious condition that can occur with junctional epidermolysis bullosa (PA-JEB). Early recognition is necessary for timely intervention, but prognosis can be serious and mortality is high.
Objective: We describe the case of a patient with PA-JEB who not only survived past infancy, but showed improvement in cutaneous blistering as she grew older.
Conclusion: With early surgical intervention, some patients with PA-JEB can survive and look forward to a favourable prognosis, with improvement of cutaneous blistering by early childhood.