Intravenous immunoglobulin (IVIg) is used in the treatment of autoimmune diseases, including immune-mediated central and peripheral nervous system disorders. This article will review the indications, proposed mechanism of actions, and administration of immunoglobulin treatment in various neuropathies, neuromuscular junction disorders, and myopathies. IVIg may have more than one mechanism of action to alter the pathogenesis of underlying neuromuscular disease. IVIg treatment has been used as a first-line treatment in Guillain–Barre syndrome, chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, and second-line off-label treatment in medically refractory cases of polymyositis, dermatomyositis, and myasthenia gravis. IVIg is a well-tolerated and effective treatment for these neuromuscular diseases. With this review article, we hope to increase clinicians’ awareness of the indications and efficiencies of IVIg in a broad spectrum of neuromuscular diseases.