We present a case of a 3-month-old female with a right nasal mass. Upon evaluation with computed tomography, magnetic resonance imaging and angiography, a large right intranasal mass extending through the cribiform plate, displacing the dura, was noted. The patient underwent a combined midfacial degloving and bifrontal craniotomy for complete resection of the tumor mass. Pathologic evaluation demonstrated a mesenchymal tumor with spindle and stellate cells from which islands of immature cartilage emerged. The spectrum of histologic features closely resembled a mesenchymal chondroid hamartoma typically located in the chest wall. It is the first reported case of a chondroid hamartoma of the head and neck in the literature to date. We examine the characteristics and treatment of this unusual tumor.