Chiari I Malformation (CM) is a congenital pathology of rombencephalon development characterized by disparity of brain structures and bony compartment in posterior fossa. The incidence of Chiari I malformation ranges from 3.3 to 8.2 in 100 000. In 80% of cases CM is combined with syringomyelia. Combination of CM and pituitary adenoma (PA) is rare condition with just a few cases described in literature. Current article represents a case of successful treatment of patient with CM and PA combination and a 5-year follow-up.