A single case of chondrogenic neoplasm arising from the base of the skull is presented and discussed. These benign and slow growing tumours in the sellar region are extremely rare and as such this article provides a useful insight and discussion for consideration. The article describes a 63-year-old man presenting with a long history of sexual dysfunction, bilateral vision loss and left nasal obstruction. Endocrine tests showed that all serum levels of anterior pituitary hormones were decreased. MRI imaging showed a large mass lesion in the saddle area, extending upwards to the dorsal sellae, bilateral cavernous sinus and suprasellar region and downwards into the sphenoid sinus and nasal cavity. The optic nerves and chaism were compressed giving a bilateral superior altitudinal visual field defect and reduced visual acuity in both eyes. This man had symptoms lasting for a six-year duration prior to diagnosis so this article highlights the need to recognise signs early to allow early detection and treatment. The patient was treated with hormone replacement and transfusion of plasma and albumin, as well as a two stage surgical procedure. Possible surgical approaches are discussed in detail in the article. The tumour was removed successfully and postoperatively the patient was stable and his visual acuity and visual field defect improved markedly. His pituitary function returned to normal except for hypothyroidism.