[Caroli's disease: literature review and characteristics of a clinical case].

The Author's report the case of the simple form of Caroli's disease, a rare malformation, and review literature pointing out the difficulty of an early diagnosis, because disease can be silent for a long time too and it is identified for complication. Today, imaging, endoscopy and interventionist radiology are the most valid tools for a correct diagnosis and treatment, above all in a case of emergency, when other therapeutic solutions are not possible.