Rhinoscleroma is a progressive chronic granulomatous disease of the upper respiratory tract that may extend to the tracheobronchial tract. It is common belief that the pathology is determined by Klebsiella Rhinoscleromatis. In the authors' opinion, the infection with Klebsiella Rhinoscleromatis may not represent the only etiopathogenic factor of the disease. Rhinoscleroma is reported in many countries, but has a peculiar social and geographic distribution, in that it assumes an endemic character only in some regions of the Middle East, West Russia, North Africa, Indonesia, Central and South America. In Europe, most of the cases are reported in Poland, Hungary and Romania. In Italy, Rhinoscleroma is almost exclusively located in the southern and island regions. Rhinoscleroma is predominantly reported in rural areas, in the presence of poor socio-economic conditions, which according to many authors would be a co-factor triggering the disease. In this article, the authors review some inconsistencies in etiology, histology and epidemiology of Rhinoscleroma. Based on the overall picture, they propose that intrinsic factors, possibly of genetic origin, may give rise to the disease, and suggest possible lines of research to distinguish between extrinsic and intrinsic factors as determinants for Rhinoscleroma.