Half the patients with Lambert-Eaton myasthenic syndrome (LEMS) have a small cell lung carcinoma (SCLC). SCLC is an aggressive tumor and survival is poor. We studied the differences between LEMS patients with and without an SCLC. Several items were identified, among which are age, smoking behavior, sex, the progression of clinical symptoms, HLA, and presence of Sox1 serum antibodies. The relationship between these parameters is not fully elucidated. Data support a role for the LEMS-related immune response in suppressing the tumor activity. We summarize the literature and present data from our Dutch cohort of 104 LEMS patients to compare the clinical and laboratory characteristics of tumor and nontumor related LEMS patients.