The clinical features of Posner-Schlossman syndrome and the various differential diagnoses are described in a 20-year-old patient. Additional to Posner-Schlossman syndrome this patient showed signs of Axenfeld’s anomaly. He also presented symptoms of a status-dysrhaphicus-like state after atrial septal defect and the ability to overstrain the distal finger joints. Posner-Schlossman syndrome, heterochromic cyclitis, anterior-chamber cleavage syndrome and ICE syndrome (irido-corneal-endothelialsyndrome: Chandler’s Cogan-Reesesyndrome and progressive essential iris atrophy) often produce similar effects at the Descemet membrane, the anterior chamber angle and the iris. Because of the same mesodermal origin of these tissues we suggest that all the diseases mentioned above are merely different clinical expressions of one main disease.