Congenital cholesteatoma is a rare, primarily pediatric disease that presents in otherwise healthy ears. Typically, this disease is found in a well-defined sac in the middle ear, making it particularly suited for removal through transcanal endoscopic ear surgery. This article reviews the ways in which endoscopy can be applied to the surgical management of congenital cholesteatoma and provides a guide based on congenital cholesteatoma stage and extent. Outcomes have shown similar rates of residual disease in total endoscopic ear surgery compared with operative microscopy.