Autoimmunity plays a major role in the pathogenesis of many neuromuscular disorders such as chronic inflammatory demyelinating polyneuropathy, Guillain-Barré syndrome, polymyositis, dermatomyositis, myasthenia gravis, Lambert Eaton syndrome, and stiff person syndrome. Although most of these disorders respond favorably to the commonly used immunomodulatory agents such as steroids, intravenous gamma globulin, plasmapheresis, and chemotherapy, some are initially refractory, whereas others gradually lose responsiveness. Therefore, alternative, selective, and novel immunosuppressive agents are used to treat these cases. Among these agents, rituximab has shown promise in some of the neuromuscular disorders with minimal side effects. Rituximab is a genetically engineered antibody that depletes CD20+ B-cells and is Food and Drug Administration- approved for treatment of non-Hodgkin lymphoma, CD20+ CLL, and rheumatoid arthritis. It carries a favorable side effects profile. However, evidence of efficacy is limited to case series and large prospective randomized controlled trials are lacking. In this article, we review and discuss the available literature on rituximab in treatment of various autoimmune neuromuscular diseases.