Benign fibrous histiocytoma is such a rare tumour that only a few cases have been reported in the literature. A patient with an apparently benign lesion of the distal radius, along with congenital dislocation of the radial head, was presented at St. John's Medical College Hospital in Bangalore, India. Pain was the chief symptom. There were histological features of non-ossifying fibroma in the lesion. Because of its unusual radiological appearance and atypical clinical course, the lesion was diagnosed as fibrous histiocytoma. In this case report, we review similar cases in the literature and discuss an unusual association with congenital dislocation of the radial head.