Simple congenital ptosis is the most common form, characterised by fatty dystrophy and fibrosis of the levator muscle, which leads to a restricted elevation in upgaze and a lid lag in downgaze. In the complicated form additional changes will be found: fibrosis of extraocular muscles, synkinesia or anomalies of the lids. Moreover, congenital ptosis is relatively often associated with refractive errors and/or disturbance of the binocular vision. Therefore a careful examination is necessary. Therapeutic management includes first prevention of amblyopia either by occlusion or by early surgical intervention. The choice of operation depends on the grade of ptosis, the actual levator function and the presence of lid anomalies as well.