Pleomorphic hyalinizing angiectatic tumor (PHAT) is a rare low-grade malignant mesenchymal neoplasm. It commonly occurs in the limbs and trunk, with limited occurrences in the head and neck region. Only five cases of PHAT occurring in the head and neck have been reported in the literature to date. The etiology of PHAT remains unclear. We described a case of nasal cavity PHAT following CARE guidelines. A 32-year-old male with PHAT originating in the nasal cavity recurred after two surgeries. Adjuvant radiotherapy was performed after the third surgery. The patient has been regularly followed up for 36months, and no tumor recurrence or metastasis has been observed. PHAT is a rare soft tissue tumor known for its local aggressiveness. Because of a high risk of recurrence, extensive resection should be implemented, possibly completed by radiotherapy if resection cannot be complete because of proximity to at-risk structures.