[Atypical course of Budd-Chiari syndrome with hydrothorax].

Budd-Chiari syndrome is a very rare condition with an incidence and a prevalence of respectively 0.8 and 1.4 per million inhabitants per year. Significant large right-sided pleural effusion without significant ascites is well-known in portal hypertension and cirrhosis, where it occurs in 5-10% of the patients. Due to the presence of endometriosis and the dominant symptom in the form of hydrothorax up to 5 l per day delayed the correct diagnosis in a case with a 33 year-old woman. Reviews of the initially performed computed tomographies could have been made shortly after admission thus avoiding long time illness and hospitalization.