Abstract
Adenoid cystic carcinoma is a malignant tumor of the salivary glands. Its presence in the nasopharynx is rare. It grows slowly but spreads locally and invades nerves. We present the case of a patient aged 62, a smoker, with adenoid cystic carcinoma of the nasopharynx, found first by otorhinolaryngological-specific symptoms and then by neurologic manifestations. MRI showed a tumor in the left posterosuperior wall of the nasopharynx with intracranial extension. Biopsy confirmed the diagnosis; therefore, the patient received combined chemoradiotherapy. The progression of the disease is marked by a distant recurrence after one year of follow-up. The challenge with this rare tumor entity is both diagnostic and therapeutic.