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Objective: To describe the neuro-ophthalmological manifestations and the functional state of the retina in patients with Steinert's disease. Methods: An observational, descriptive study was carried out on a series of 29 patients, from January 18, 2016 to January 15, 2018, in the Neurophthalmology service at Prof. Rafael Estrada González Neurology and Neurosurgery Institute. Demographic, clinical variables, as well as functional electrophysiological evaluation variables of the retina were taken into account. Univariate and multivariate statistical analysis was performed. Results: The mean age of the patients was 37.69 years (± 11.48); the most frequent age group was 40 to 49 years (44.8%) and the composition was similar in terms of sex. Mean visual acuity was 87.2 VAR (± 2.842), and 74.1% considered good, as was color vision (72.4%), contrast sensitivity at 1 meter (63.8 %) and 3 meters (62.1%). Ocular hypotension (82.8%), cataracts (58.64%), palpebral ptosis (51.7%), ophthalmoparesis (34.5%), epiretinal membranes (12.1%) and maculopathies (8.64%) were detected. The mesopic electroretinogram was altered in 75.9% of the patients and the electroculogram in 39.7%. Conclusions: Structural and functional ophthalmological alterations were found in patients with Steinert's disease. The evident affectation, detected in the visual electrophysiology tests, suggests an important dysfunction of the photoreceptor layer of the retina and its pigment epithelium (greater in the former), unlike that described historically in this disease. [ABSTRACT FROM AUTHOR]