Objective: To study the clinicopathologic features of tuberous sclerosis complex (TSC) occurring in children with medically intractable epilepsy. Methods: The clinical, radiologic and pathologic features of tuberous sclerosis complex occurring in 12 children with intractable epilepsy encountered during the period from 2011 to 2016 in the Epilepsy Center of Haidian Hospital were retrospectively reviewed. Results: All patients including 8 boys and 4 girls had medically intractable seizures. The mean age of seizure onset and disease duration were 3.28 years and 5.32 years respectively. Multiple lesions were existed in single lobe or bilateral cerebral hemisphere. Giant neuron, dysmorphic neurons and balloon cells were pathological characteristic. Seizure outcome after the epilepsy operation revealed 4 children (33.3%) had an Engel grade Ⅰ, 2 kids (16.7%) had an Engel grade Ⅱ, 5 children (41.7%) had an Engel grade Ⅲ, and 1 patient (8.3%) was lost. Conclusion: The children with refractory epilepsy suffered the onset of the disease early and long duration of the disease. Lesion of brain tissue has pathological characteristics. There was a certain correlation between the effect of surgery and the range of the lesion. The effect of surgery was poor in children with extensive lesions.