Central nervous system (CNS) involvement in chronic lymphocytic leukemia (CLL) is rare, and spinal cord infiltration as a presenting manifestation has only rarely been described. We present the case of a 65-year-old man with CLL, initially diagnosed at the age of 54, who had not received prior treatment. He presented with a six-week history of thoracic and epigastric pressure. Mild ataxia was found in the clinical exam. Clinical evaluation revealed leukocytosis (163 G/L; normal range: 4-10 G/L). MRI of the spine showed a mildly enhancing, T2 hyperintense central lesion extending between C3 and T8. There was a pleocytosis of 105 /micro/l consisting of neoplastic B-lymphocytes. The bone marrow biopsy diagnosed a relapse of CLL, and the patient was started on ibrutinib, which had a positive effect. This case highlights spinal cord infiltration as a rare initial manifestation of CLL relapse, presenting with non-specific symptoms such as thoracic and epigastric pressure and mild spinal ataxia.