Background: Lymphoma-associated incomplete Miller-Fisher syndrome is very rare. Case Characteristics: An 11-year-old boy who initially presented with headache, left ptosis, diplopia and weakness. Neurologic examination indicated left sided ptosis with ophthalmoplegia. Observations: Cerebral imaging and cerebrospinal fluid examinations were normal. Magnetic resonance imaging of the abdomen showed a mass lesion in the ileal loops. A bone marrow biopsy showed infiltration by Burkitt's lymphoma. Message: Burkitt lymphoma may present with incomplete Miller Fisher syndrome.