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Purpose To describe a pattern of combined exotropia and hypotropia in patients with unilateral high myopia and to suggest a surgical approach for their management. Methods In this observational study of 13 patients presenting with unilateral combined exotropia and hypotropia with high axial myopia in the deviating amblyopic eye, cycloplegic refraction, visual acuity, ocular motility, and orbital imaging findings were evaluated. For patients who had undergone surgery, the intraoperative findings and their surgical outcome were also analyz