Hailey-Hailey disease or Chronic Benign Familial Pemphigus is probably rare in this region and to date there is no case report from Malaysia. I report here a Malay patient with this disease but with no family history and who was believed to be suffering from Atopic Eczema for several years. Dapsone effectively controlled the disease in this patient. The clinical features, histology and the therapy are discussed.