Changes in ocular anatomy and topography that occur due to congenital development anomalies are often of complex, composite nature. Such pathologies are accompanied by not only refractive errors, but also changes in eye’s macrostructure. Careful analysis of the changes is necessary to better understand the clinical picture. Purpose — to study the specifics of structural and morphological changes of the eye in certain ocular congenital pathologies and in some orphan diseases using the results of three-dimensional ultrasound analysis and digital acoustic imaging. Material and methods. The study included 60 patients (119 eyes) with congenital ocular changes, some of which are registered as independent orphan diseases, as well as with systemic manifestations including congenital changes in eye membranes, lens and the vitreous body. The examination involved three-dimensional scanning followed by digital processing of the imaging data obtained using general medical ultrasound systems Voluson 730 Pro Kretz and Voluson E8. Results. Digital ultrasound technologies significantly expand the scope of data on eye morphology in various congenital changes obtainable in vivo. Conclusion. The advantage of this investigation method is the possibility to reliably detect congenital spatially complex morphological deformations in the eye tissues, which in some cases are impossible to observe with other methods.