The basioccipital skull base is an extremely rare site for meningoencephalocele. The herniation of the brainstem or part of it through a basioccipital defect is even rarer. We report a case of rhombencephalomeningocele associated with a tethered dermoid. An extensive literature search revealed no such case report of basioccipital cranial dysraphism so far presenting with cranial nerve palsy.