This case study was to examine the effect of the sensory integration therapy on the Goldenhar syndrome child with tactile defensiveness, developmental delay and occupational performance difficulties. The child was 7.10 year old boy. For intervention, four evaluations were used and the results are as follows. First, in Canadian Occupational Performance Measure showed occupational performance goal as reading and writing in upright position. Second, in Short sensory profile and sensory history, significant differences between sensory sensitivity and tactile processing. Third, in Developmental Profile-3 showed delay. Fourth, in Bruininks-Oseretsky Test of Motor Proficiency-2 showed below average. After intervention, the results of which showed a positive changes. Therefore, this case study is found that the sensory integration therapy is effective in the Goldenhar syndrome child with tactile defensiveness and developmental delay.