Chordomas are rare tumors which originate from the remnants of the notochord. These tumors are locally aggressive and have a predilection for the ends of the axial skeleton. An important prerequisite for optimal management of these tumors is a correct preoperative diagnosis. The present case is the first report of the use of endoscopic ultrasound to obtain transrectal fine needle aspiration biopsy of a presacral chordoma. A review of the prior computer tomography (CT) scans allowed us to calculate the tumor volume doubling time (18.3 mo). Transrectal biopsy of chordomas is controversial, however we believe that such concerns are not justified. Chordomas are rare tumors which originate from the remnants of the notochord. These tumors are locally aggressive and have a predilection for the ends of the axial skeleton. An important prerequisite for optimal management of these tumors is a correct preoperative diagnosis. The present case is the first review of the use of endoscopic ultrasound to obtain transrectal fine needle aspiration biopsy of a presacral chordoma. A review of prior computer tomography (CT) scans allowed us to calculate the tumor volume doubling time (18.3 mo). Transrectal biopsy of chordomas is controversial, however we believe that such concerns are not justified.