The association between Amyotrophic Lateral Sclerosis (ALS) and Neurocysticercosis (NCC) has rarely been described in literature. We describe a patient with both disorders. NCC was diagnosed in a 55 year-old male patient with positive CT scan and cerebrospinal fluid (CSF) immunoassay tests. After Praziquantel treatment, he developed slurred speech, bradylalia and periods of speech arrest. He also demonstrated bulbar, upper and lower motor neuron symptomatology. Electromyography supported definite ALS diagnosis, and brain MRI confirmed the presence of NCC in motor cortices. Although the association between NCC and ALS may have occurred by chance, we hypothesize that autoimmune, apoptotic and circulatory mechanisms contributed in the development of ALS in this patient.