Objective To observe the morphological characteristics of congenital optic disc pit with maculopathy, the natural de-velopment, and changes after laser photocoagulation. Design Retrospective case series. Participants Twelve cases with congenital optic disc pit. Methods Records of 12 patients with congenital optic disc pit with maculopathy were reviewed. Clinical examination includes optical coherence tomography (OCT), color fundus photography, and fluorescein angiography (FA). The data was analyzed with the exist-ing theory of pathogenesis of the disease. Main Outcome Measures Visual acuity and morphology of macupopathy. Results All the patients were noted to have serous maculopathy associated with optic disc pit. Serous detachment of neuro-retina was found in two pa-tients, schisis of neuro-retina in two patients, and both serous detachment and schisis of nearo-retina were observed in other patients. Two patients were associated with choroidal coloboma. Four patients were treated with laser photocoagulation, in which 3 patients had vision improved. Conclusions Schisis and detachment of neuro-retina are the important morphologic changes of congenital optic disc pit with maculopthy. Proper understanding of the relationship between the development of the disease and these changes will be helpful to study its pathogenesis. Patients may benefit in part from laser photocoagulation. (Ophthalmol CHN, 2009, 18: 233-236)