Objectives: To report the case of a congenital nasal chondromesenchymal hamartoma in a oneyear-old female and review the literature, identifying problems encountered in confirming the diagnosis and in treatment of this patient. Methods:Design: Case Report Setting: Tertiary Public General Hospital Patient: OneResults: A one-year-old female with an intranasal mass noted at birth and with subsequent unilateral maxillary enlargement is described. Computed tomography showed calcifications and erosion of adjacent bony structures. Histopathology and immunohistochemistry of an intranasal biopsy were interpreted as chordoma, a malignant tumor. Following surgical excision, the final histopathologic diagnosis was chondroid hamartoma. Conclusion:Only 20 cases of nasal chondromesenchymal hamartoma have been reported in the literature worldwide. These tumors may present clinically, histopathologicaly and radiologically as malignant tumors and may mislead even the experts. The whole clinical picture should be taken together to avoid misdiagnosis as a malignancy and to facilitate appropriate management.

Objectives: To report the case of a congenital nasal chondromesenchymal hamartoma in a one-year-old female and review the literature, identifying problems encountered in confirming the diagnosis and in treatment of this patient. Methods: Design: Case Report  Setting: Tertiary Public General Hospital Patient: One Results: A one-year-old female with an intranasal mass noted at birth and with subsequent unilateral maxillary enlargement is described. Computed tomography showed calcifications and erosion of adjacent bony structures. Histopathology and immunohistochemistry of an intranasal biopsy were interpreted as chordoma, a malignant tumor. Following surgical excision, the final histopathologic diagnosis was chondroid hamartoma. Conclusion: Only 20 cases of nasal chondromesenchymal hamartoma have been reported in the literature worldwide. These tumors may present clinically, histopathologicaly and radiologically as malignant tumors and may mislead even the experts. The whole clinical picture should be taken together to avoid misdiagnosis as a malignancy and to facilitate appropriate management.  Keywords: nasal chondromesenchymal hamartoma, nasal masses in infancy, nasal chondroid lesions