Objective To investigate pseudoptosis with a vertical deviation of the extraocular muscles and to evaluate different outcomes based on imaging and clinical findings. Methods This was a retrospective study that included 12 patients who were treated between August 2007 and October 2009. Preoperative ophthalmologic examination and orbital CT scan were performed. The choice of surgical procedure was based on the results of the clinical evaluation and CT imaging.Results Of the patients, 75%(8/12) showed significant changes in their extraocular muscles. In patients' coronal images, the maximum cross-sectional area of the superior rectus-levator complex in the affected eyes became thinner than that of the contralateral eyes [(15.11±7.11)mm2 vs (24.93±6.43)mm2, P＜0.01]. The vertical misalignment was completely corrected in all patients. Pseudoptosis totally disappeared in 10 patients and partially improved in 2 patients. Conclusion Either congenital hypoplasia or paresis of the superior rectus-levator complex in the affected eye may be the main cause of pseudoptosis with vertical deviation. Surgical procedures for pseudoptosis with vertical deviation must be individualized according to the clinical evaluation and imaging. CT imaging of the extraocular muscles may provide clinically valuable information for choosing the appropriate surgical procedure.