Abstract
Objective: This article aimed to gather, evaluate and synthesize studies on the symptoms and therapeutic difficulties of patients with Caroli Disease (CD), as well as to elucidate the diagnosis and what are the possible definitive treatments. Methods: Review of 20 scientific publications indexed in the following electronic databases: PubMed, SciELO and Science direct. The revised publications address articles from the last 10 years of a descriptive and qualitative methodological nature. Results: The diagnosis of CD consists in demonstrating the communication between the dilated segments of intrahepatic bile ducts and the rest of the bile system. A detailed clinical history, precise physical examination and confirmation of the imaging exam, which can be performed by ultrasound, where the dilation of the intrahepatic bile ducts is observed, computed tomography, observing the "central point" sign with cystic dilation imaging or by cholangiopancreatography by magnetic resonance imaging, the latter being highly specific for CD. Final Considerations: Although rare, this pathology significantly affects the quality of life of the patient, due to the systemic repercussions arising from infectious diseases and their complications. Due to its severity, early diagnosis becomes crucial, and therefore the knowledge of the health professional about the signs and symptoms of this pathology is fundamental.