Objective To observe the characteristics of three-dimensional optical coherence tomography (3D-OCT) in congenital zonal choroidal dystrophy (CZCD) patients. Methods 3D-OCT data from 11 patients (13 eyes) who were diagnosed with CZCD was retrospectively analyzed and combined with fundus photography. Results The characteristics of 3D-OCT in CZCD patients were as follows: ①The reflex curve changes of the retinal pigment epithelium (RPE) and choroid band as well as the notch were observed in 9 eyes of 8 patients. And the notches were all located on the temporal side of the fovea. The separated layer of the retina revealed by 3D-OCT showed that there was an obvious concavity of the RPE corresponding to the notch. ②RPE was discontinuous in 1 eye. The separated layer of the retina revealed by 3D -OCT showed that there was an obvious corresponding concavity in the RPE and choroid band. ③The RPE and choroid band were absent in the macular area in 2 eyes. There was a hollow in the RPE and choroid band under the fovea. ④A higher reflex was observed in the choroid where the RPE and choroid band had dystrophy. This is the result of the fibration of the choroid structure. Conclusion There are different degrees of change in the RPE and choroid membrane of the macular area. These changes include a change in the reflex curve, a discontinuity of the RPE and the absence of the RPE. All of this is the result of an anormaly of the embryonic fissure. It is difficult to discover where there is no complication. Therefore, we suggest that 3D-OCT should be performed routinely when a patient's visual acuity is not normal even if there is no evidence of eye disease. It may be diagnosed as CZCD by 3D-OCT.