Objective To investigate the misdiagnosis causes and clinical diagnosis of congenital cholesteatoma of temporal bone in children.Methods The clinical data of a child with congenital cholesteatoma of the temporal bone misdiagnosed with Bell facial palsy were reviewed, and the clinical and imaging characteristics of congenital cholesteatoma of the temporal bone were discussed. Results? CT scan of the temporal bone of this patient showed intraosseous soft tissue image in the left temporal bone, expansive growth, destruction of the ossicle, involving the semicircular canal, superior labyrinth air chamber, horizontal segment, and labyrinth facial neural tube. MRI showed a large amount of iso-hyposignal on T1WI and iso-hypersignal on T2WI+FS in the left tympanum. The lesions extended inward to the vestibule, superior semicircular canal, horizontal semicircular canal and ampulla margin. Preoperative diagnosis: congenital cholesteatoma. Mastoid radical cure + tympanum molding art + facial nerve decompression art, postoperative recovery is good.Conclusions Congenital cholesteatoma of the temporal bone in children is rare in clinic and easily misdiagnosed as other diseases. Clinical symptoms and imaging methods are the main methods for diagnosis of congenital cholesteatoma of the temporal bone in children. At the same time, it is necessary to improve the understanding of the disease to reduce misdiagnosis