Pierre Robin sequence (PRS) is a rare congenital disorder that may be associated with pathological myopia and other ocular abnormalities.[3] The article describes the case of a 4-year-old boy with PRS syndrome, in whom pathogenic variants were identified during genetic tests.[3] The patient had high myopia in both eyes with a best-corrected visual acuity of 0.12 in the right eye and 0.1 in the left eye, while the axial length of the eyes was 31.38 mm in the right eye and 32.13 mm in the left eye.[3] Ophthalmic examinations revealed bilateral medial entropion of the upper lid with trichiasis and a mosaic fundus appearance with macular atrophy.[3] The boy underwent bilateral posterior scleral strengthening and upper lid entropy correction, as well as low vision rehabilitation interventions.[3] At 35-month postoperative follow-up, visual acuity improved to 0.3 in the right eye and 0.12 in the left eye, while the growth of the axial length of the eyes slowed and the condition of the fundus remained stable.[3] The case demonstrates that multidisciplinary management, including surgery and long-term follow-up, is critical even in cases where pathogenic genetic variants are not identified.[3]