The purpose of this paper is to presentWegener granulomatosis manifesting in its periocular form. Review with new developments in understanding of the etiopathogenesis, clinical and laboratory findings as well as therapy modalities are shown through a series of patients gathered in collaboration with colleagues from UMC Utrecht, Netherlands. In the period from 1992 until 2004 the group of 54 patients with established diagnosis of Mb. Wegener were observed. 13 patients developed periocular form but only 2 presented it as initial symptoms. Lacrimal stenosis and orbital infiltration were predominant periocular symtoms while nasal manifestations were predominant systemic symptoms of the disease. Different treatment modalities were employed showing that orbital disease is difficult to treat in spite of satisfying systemic answer to immunosuppressives which calls for alternative solutions.