Objective:The aim of this study was to summarize clinical features of Mowat-Wilson syndrome (MWS) in Chinese children and its associated characteristics with epilepsy. Methods: The clinical and genetics data of MWS patients admitted to the Maternal and Child Health Hospital of Guangxi Zhuang Autonomous Region were analyzed retrospectively. We also collected and analyzed all the previous reports of MWS in Chinese children. Results: Twelve cases of MWS were used in this study (4 males, 8 females), including our reported 2 cases. The age of seizure onset ranged from 6 to 48 months. Of these, 50% of the cases initially presented with febrile seizures, and 7 cases (58.3%) exhibited focal seizures. Valproate (VPA) was used alone or in combination in 7 of the cases, with 3 cases showing effective seizure control when subjected to monotherapy. One of our reported cases showed electrical status epilepticus during sleep (ESES), and an electroencephalogram performed after hormone treatment, showed some improvements in the patient. Brain magnetic resonance imaging revealed structural abnormalities in three quarters of the 9 cases with the rest presenting as normal.Conclusion: Epilepsy in MWS tends to initiate with febrile seizures and exhibit no obvious correlation with brain structural abnormalities. VPA is the most frequently prescribed drug and it has proven to be an effective anti-seizure medication in MWS, while hormone treatment may improve ESES.