Primary pleomorphic adenoma (PA) of the parapharyngealspace (PPS) is relatively rare. It shows a real diagnostic and therapeutic challenges. We report a case of a 25-year-old male, who was consulting for dysphagia and sleep apnea syndrome evolving over eight months. Physical examination revealed a submucosal mass arinsing from the lateral wall of the left oropharynx, narrowing its lumen. Imaging revealed a heterogeneously enhancing tumor in the left PPS, separated from the deep lobe of the parotid, measuring 60/47 mm. The patient underwent combined transcervical and transoral approach, complete tumor removal was insured. Histopathologic study confirmed the diagnosis of PA. No postoperative complications and no recurrence were noticed during 24 months of follow up. Primary PA arising in the PPS is of rare occurrence. The diagnosis seems difficult and remains based on histopathological examination. A suitable surgical approach must be required to insure complete and safe removal of this tumor.