Bizarre parosteal osteochondromatous proliferation of bone (BPOP) is a beni
gn lesion that is occasionally misinterpreted as a malignant process. The o
riginal reports described lesions exclusively in the hands and feet. Howeve
r, subsequent reports have included additional sites in the long bones, sku
ll, and maxilla. The differential diagnosis of BPOP includes numerous benig
n and malignant lesions. The benign differential diagnosis includes osteoch
ondroma and reactive processes. The most important malignant differential d
iagnosis is parosteal osteosarcoma. We present a case of an 11-year-old boy
with recurrent BPOP and review the literature. We discuss the differential
diagnosis and pathogenesis of the lesion.