The extremely rare condition of bizarre parosteal osteochondromatous proliferation (BPOP) was first described in 1983 by the pathologist Nora, and a few more than 100 cases have since been reported. The lesion is defined as a reactive heterotopic ossification and is mostly found in the hands or feet of adults in the third decade of life, although it has also been described in long bones and in other age groups. A high rate of local recurrence of up to 50 % has been noted, but the lesion is benign and does not metastasise. An association with chromosomal rearrangements has recently been described. We here report the case of a 12-year-old girl with a BPOP at the second metacarpal bone, thus at an unusual age. The lesion was marginally resected after biopsy, but recurred locally within 2 years, resulting in subtotal resection of the second metacarpal bone, autologous fibula grafting and temporary external fixation. The clinical, plain radiographic and MRI appearance of the lesion and the prominent histological findings are described, and the difficulties in establishing the correct diagnosis in cases of BPOP are discussed.