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AIM: To investigate the clinical features of fundus diseases of familial congenital nystagmus. <p>METHODS: Totally 40 eyes of 20 nystagmus patients from 3 congenital nystagmus families were enrolled in our study. The eye position, refractive error, anterior segment and fundus, including fundus photograph, optical coherence tomography(OCT), and visual evoked potential(VEP)were performed on them. <p>RESULTS: There were 8 patients in Family A, all of which were horizontal nystagmus, in that 6 cases of strabismus(2 of which combined with compensatory head posture), 2 cases of nuclear cataract, 2 cases of posterior polar cataract, 1 case of retinopathy of prematurity, 2 cases of severe ametropia. There were 6 patients in Family B, all of which were horizontal nystagmus, in that 3 cases of albinism(2 of which combined with macular hypoplasia), 2 cases of severe ametropia, 1 case of achromatopsia, 1 case of nuclear cataract. There were 6 patients in Family C(5 of which were horizontal nystagmus and 1 rotatory nystagmus), 2 cases of Leber congenital amaurosis, 1 case of familial exudative vitreoretinopathy, 2 cases of posterior polar cataract, 1 case of iris atrophy, 2 cases of strabismus. <p>CONCLUSION: We have to do detailed examinations on patients suffered from familial congenital nystagmus to understand its causes and to improve their visual functions as well as possible.