Extraintestinal manifestations (EIM) of inflammatory bowel disease (IBD) occur rather frequently and may be found in up to 30% of patients. However, surprisingly few randomized, controlled studies have been conducted that were specifically aimed at the treatment of EIM of IBD patients. Therefore, most therapies of EIM are empiric or deduced from studies in populations with other type of patients. EIM may be associated with active IBD. Treatment of active IBD is, therefore, the mainstay of treatment of EIM. Lifestyle modification as a means of therapy is a recent subject of study in chronic conditions, such as IBD. Based on epidemiologic and experimental findings, EIM of various tracts can be modified by optimizing alimentary intake, refraining from sedentary lifestyle, and adapting smoking habits. Not many new drugs for treatment of EIM have been developed during the past few years; the role of infliximab has been extended in particular in Crohn's disease-related EIM. Careful consideration of prescribed drugs remains necessary due to potential interaction with the course of IBD.