Alveolar soft part sarcoma is a rare soft tissue sarcoma with a predilection for adolescents and young adults that tends to occur in the extremities. It is a well-differentiated and chemo-resistant solid tumor. Lingual alveolar sarcoma can occur at an early age and appears, in this case, to be chemo-sensitive. We report the case of a child with an ASPS of the tongue and the mouth floor with lung metastasis, never yet published in Morocco, and a review of the literature. Alveolar soft part sarcoma of the tongue and the mouth floor was diagnosed in a 13-year-old boy. He had a painful, occasionally bleeding, medio-lingual swelling, spanning the mouth floor and the pharynx, in progression for a year, complicated with dysphagia and dyspnea requiring emergency tracheotomy. He underwent a surgical excision of the mass, and then was treated by chemotherapy. The child died from hemorrhagic shock and worsening of the respiratory distress. ASPS should be included in the differential diagnosis of head and neck masses in children since early detection and treatment are essential. In particular, lingual alveolar sarcoma diagnosed at an early age seems to be chemo-sensitive.