Osteosarcomas have been reported as arising in a number of extraosseous pri
mary sites, most commonly in the retroperitoneum and the muscles of the thi
ghs and limb girdles, but also in a variety of other organs. We present a c
ase arising in the penis, which we believe to be only the fifth reported in
the literature and the only documented long-term survivor. Careful histopa
thological analysis and surgical management remain of key importance in the
management of all sarcomas arising in soft tissue.