Carpal tunnel syndrome can be the result of a wide variety of etiologic factors. Compressive causes are rare and tumors causing compression are even rarer. We present a case with a carpal tunnel syndrome caused by an intraneural lipoma and an arteriovenous malformation concomitantly. The lesions were excised, and the patient recovered with full hand function and entire disappearance of acroparesthesia. To our knowledge, this is the first case report of carpal tunnel syndrome caused by an intraneural lipoma and an arteriovenous malformation concomitantly.