In this study, the case of a 58-year-old man with a 2-month history of left chin paresthesia with difficulty swallowing and chewing, and dysphonia is reported. He had an absence of the gag reflex, unilateral palatal palsy and deviation of the tongue upon attempted protrusion with unilateral atrophy. Magnetic resonance imaging of the skull base revealed a tumoral infiltration of the left side of Meckel's cave, involvement of the clivus with extension into the cavernous sinus and signs of focal leptomeningeal infiltration. The patient was eventually diagnosed with generalized Burkitt's lymphoma. In this study, the authors suggest an initial diagnostic workup that includes a computed tomography scan of the mandible and a magnetic resonance imaging of the skull base searching for malignancy in patients with isolated numb chin syndrome, a rare disorder often associated with cancer, especially breast cancer and lymphoma, with mandibular metastases, leptomeningeal seeding and metastases of the base of the skull explaining the origin of the syndrome in most cases.