Chordomas are slow growing neoplasms arising from notochordal remnants of the axial skeleton. The second most common site for chordomas, after the sacrococcygeal region, is the base of the skull. We describe one case of sellar and suprasellar chordoma found in a 44 year old female, with tumoral syndrome, bitemporal hemianopsia and secondary amenorrhea. Skull X-ray showed an enlarged sella turcica with destruction of the dorsum and impressive intra- and suprasellar calcifications, reason why a craniopharyngeoma was initially suspected. MRI depicted a voluminous and expansive solid tumor mass, accompanied by destruction of the sellar base and temporal bone on the left side. The lesion was compressing the optical chiasm and the third ventricle on the left side. Hormonal investigations showed corticotroph and somatotroph deficiency (morning plasma cortisol of 45 ng/ml, basal GH of 0.2 ng/ml both insufficiently stimulated by insulin-induced hypoglycemia test to 56 ng/ml for cortisol and 1.1 ng/ml for GH) as well as thyrotroph (basal TSH of 0.19 mIU/l, stimulated only to 1.66 mIU/l at TRH test 500 microg iv in the context of low total T4 5.2 mg/l) and gonadotroph deficiency (low basal FSH, of 1.5 mIU/ml, in the context of low plasma oestradiol, of 29 pg/ml). Basal prolactin was moderately increased (79.6 ng/ml) and further stimulated by the TRH test (to 117.4 ng/ml), suggesting pituitary stalk disjunction rather than tumoral secretion. The patient was submitted to transfrontal surgery under intravenous glucocorticoid protection. The anatomopathological investigation set the final diagnosis of chordoma, due to the presence of physalyphorous cells. After surgery the visual field broadened, but the patient developed irreversible diabetes insipidus. The patient was hormonally substituted and submitted two years later to telecobaltotherapy due to the growth of tumor reminiscence and re-narrowing of the visual field. Her evolution eight years after the diagnosis is stable.