AIM: To investigate the characteristics of the imaging of extraocular muscles in congenital double elevator paralysis(CDEP) and to evaluate different surgical outcomes based on imaging and clinical findings. METHODS: Sixteen patients were included in this retrospective study, from April 2006 to July 2009. Pre-operation ophthalmologic examination and orbit CT scan were performed. The choice of surgical procedure was based on the result of clinical evaluation and CT imaging. RESULTS: It showed that 81.3%(13/16) patients showed significant changes in extraocular muscles. In 68.7%(11/16) patients coronal images, the maximum cross-sectional area of superior rectus complex in affected eye became thinner than that of contralateral eye (9.95±2.82mm2 vs 24.52±5.42mm2,P<0.01),while no significant difference was found in inferior rectus(21.20±3.82mm2 vs 23.41±4.73mm2, P>0.05). In two patients, the maximum cross-sectional area of inferior rectus in affected eye became thicker than that of contralateral eye. The vertical misalignment was completely corrected in 14 patients and partially corrected in 1 patient. Psuedoptosis totally disappeared in 8 patients and partially improved in 7 patients. CONCLUSION: Either congenital hypoplasia or paresis of superior rectus muscle in affected eye may be one of the main reasons of CDEP. Surgical procedures for CDEP must be individualized according to clinical evaluation and imaging. Extraocular muscle CT imaging may give clinically valuable information for appropriate surgical planning.