Objective: Different clival lesions have been reported in the literature previously. The lymphangioma reported in the present case seems to be the first lesion localized in the clivus.
Clinical presentation: The patient presented with a history of diplopia and headache. Imaging studies showed a lytic lesion at the cranial base.
Intervention: Transsphenoidal excision of the lesion resulted in total resolution of the patient's complaints.
Conclusion: Lymphangioma must be kept in mind for the differential diagnosis of lytic lesions of the cranial base. Surgical removal leads to immediate improvement of the symptoms.