Adamantinoma is a rare low-grade malignant epithelial bone tumour.

We report a case of an expansile, osteolytic mid-diaphyseal tibial lesion found in a 12 year-old girl. An initial histological diagnosis of basaloid-type adamantinoma was made. Following excision, further histology demonstrated basaloid cells and acellular matrix focally surrounded by osteoclast giant cells with calcium deposits, features consistent with pilomatrixoma. Several histological variants of adamantinoma have been documented; this case details a previously unreported histological adamantinoma variant – pilomatrixoma-adaminatinoma.