Chordomas are rare tumors believed to be originated from notochordal remnants. Of all intracranial neoplasms, the incidence of cranial chordomas is less than 1%1. The youngest patient with an intracranial chordoma reported in the literature was a newborn in the first few days after birth9. Intracranial chondroma are more predominant in males compared to females1. The clinical features of intracranial chondroma in the pediatric age group commonly include increased Intracranial pressure, lower

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... nial nerves palsy, and torticollis2. There is no optimum treatment, however, surgical resection of the tumor followed by radiation therapy reported successful outcome3. This is a case of a poorly differentiated clival chordoma of a 23-month old boy. The clinical features, pathological, radiological findings, and surgical technique are discussed with an elaboration of the current review of the literature of clival chordoma in the extreme young age group. To the best of our knowledge, this is the youngest age of clivus chordoma reported in Saudi Arabia.