RESUMO
Hamartoma designates a tumor-like, non-neoplastic malformation, or in-born error of tissue development, often with an abnormal mixture of tissue indigenous to the region. In the head and neck, epithelial and mesenchymal hamartomas are uncommon. We present the case of a 9-year-old boy with a huge hamartoma of 800ml (cm(3)) volume of the right retro- and parapharyngeal region. It had fully occluded the nasopharynx, indented the base of the tongue and left a minimal airway passage transorally. Food uptake was severely hampered by the lesion. The patient underwent a combined transoral and external approach for complete resection of the tumor. This is the first reported case of a retro- and parapharyngeal hamartoma with metaplastic bone formation in the literature and further more, the largest hamartoma in head and neck ever reported in this age group.