Although multiple synchronous malignancies of the same histological type have been described frequently in the upper aerodigestive tract, this is a less frequent phenomenon when dealing with tumors of different lineage. We present a case of a man who developed simultaneously an oral squamous cell carcinoma and chronic lymphocytic lymphoma of the palate without any previous risk factors. To the best of our knowledge, this is the first case well documented in the literature of synchronous oral squamous cell carcinoma and chronic lymphocytic lymphoma of the palate in the same sample. The presence of multiple primary malignancies of different histological types not only complicates the treatment but also worsens the prognosis of the patient.