Acute hepatic porphyrias are the diseases dependent on the congenital metabolic defects of the hem biosynthesis. The diagnosis of porphyria only on the grounds of clinical picture is extremely difficult as it is presented by the variety of symptoms, often imitating other diseases. In each case the diagnosis must be confirmed by the biochemical and enzymatic investigations. In the paper we described different clinical courses of the acute hepatic porphyrias. The acquaintance with the symptoms of porphyrias enables to decrease the number of complications connected with diagnostic and therapeutic errors what is still a serious economic problem.