ObjectiveTo explore the clinical manifestation, diagnosis, treatment and prognosis of infantile spasm complicated with craniostenosis.MethodsA case of infantile spasm complicated with craniostenosis in the Department of Neurology of Qilu Children's Hospital in December 2017 was reviewed with the literature. The clinical manifestations, diagnosis, treatment and prognosis of infantile spasm with craniostenosis were analyzed.ResultsThe proband infantile spasms and craniostenosis was diagnosed by clinical, imaging examination and VEEG. Epileptic attack was prevented and craniostenosis was corrected by hormone shock therapy (corticotrophin was administered for 14 days, followed by topiramate)and surgical treatment (cranial cap reconstruction was performed), and good clinical prognosis was obtained.ConclusionThis case was the first reported case of craniostenosis with infantile spasm in China, and compared with the foreign treatment method, better treatment method and the operation opportunity were obtained. Which has a significant effect on the clinical treatment of infantile spasm complicated with transcranial disease.