Objective To analyze and summarize the clinical features and types of strabismus in pediatric patients with neurologic diseases. Methods The clinical data of pediatric patients with neurologic diseases who visited the Ophthalmology Department of Xinhua Hospital from January 2010 to March 2011 were retrospectively analyzed. The data included sex， age， type of strabismus， refractive errors， ocular fundus and visual acuity. Twenty-three patients were diagnosed as strabismic children with neurologic disease. Among them， twelve were males （52%） and eleven were females （48%）. Results They were diagnosed at an average age of 3.8±2.1 years （range from 6 months to 8 years）. Eighteen patients （78%） had exotropia with a horizontal strabismic angle ranging from -30～-120 PD （mean -73.6±30.6 PD）； 5 cases （22%） had esotropia， 1 had primarily accommodative esotropia and 4 had nonaccommodative esotropia with a horizontal esotropia strabismic angle ranging from +25～+35 PD （mean +29.0±4.2 PD）. Five patients had an A-pattern and 4 patients had a V-pattern； 4 patients had DVD and 1 had latent nystagmus. Visual acuity measurements were possible in only 7 patients and their best corrected visual acuities were all below 0.8. No one had stereoscopic vision before the surgery. Fifteen patients （65%） had associated cerebral palsy， 3（13%） had hydrocephalus， 3（13%） had mental retardation， 1（4%） had epilepsy and 1 patient （4%） had associated hypoxic ischemia encephalopathy. Twenty-two patients agreed to have strabismus surgery. The median follow-up was 9.6±3.6 months （range from 3 to 15 months）. Twenty patients （91%） returned to a normal primary eye position and 5 patients recovered different degrees of binocular vision. Conclusion Strabismus of congenital exotropia was found to be common in patients with neurologic diseases. Cerebral palsy was the most common neurologic disease. Amblyopia or destruction of binocular vision was found in the great majority of the cases.