Neurosarcoidosis is an uncommon clinical entity, even in patients with systemic sarcoidosis. Its presentation is widely varied but longitudinally extensive transverse myelitis secondary to neurosarcoidosis has only rarely been described. We report the case of a patient with pulmonary sarcoidosis and congenital immunosuppression who developed longitudinally extensive transverse myelitis secondary to neurosarcoidosis following splenectomy. Diagnosis was based on history, inflammatory changes on MRI, and cerebrospinal fluid findings. Standard therapy with intravenous steroids was unsuccessful. Only more novel treatment with Infliximab allowed improvement.