Parapharyngeal space tumors are uncommon, most are salivary gland tumors (50%), and they are often pleomorphic adenomas. Neurogenic tumors are the second most common primary tumors of all neoplasms in the parapharyngeal space (30%). Angiomyxolipoma is a very rare form of lipoma. Up until now, only 12 cases located on different sides of the body were reported. Presented here is a case report of a patient with an angiomyxolipoma of the parapharyngeal space. A 17-year-old boy had a slowly growing, painless mass on the left side of the neck. During the oropharyngeal examination, medial displacement of the left tonsilla palatina was observed. Magnetic resonance imaging findings were of a 6 × 5.5 × 3-cm "dumbbell"-shaped parapharyngeal mass. A fine needle aspiration of the mass showed no specific histopathology. The patient underwent a transparotid-transcervical approach for the excision of the tumoral mass under general anesthesia. Although an angiomyxolipoma is a rare form of lipoma, it is a pathology that should be kept in mind for the differential diagnosis of parapharyngeal tumors.