The types of neuromuscular diseases associated with human immunodeficiency virus (HIV) infection are described. Our classification includes: (1) six subtypes of peripheral neuropathies--namely, acute Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, mononeuritis multiplex, an axonal, predominantly sensory, painful polyneuropathy, a sensory ataxic neuropathy due to ganglioneuronitis, and an inflammatory polyradiculoneuropathy presenting as cauda equina syndrome; (2) inflammatory myopathies (e.g., polymyositis); and (3) other less common neuromuscular manifestations, such as type II muscle fiber atrophy and nemaline myopathy. Although the exact incidence of clinical and subclinical neuromuscular diseases in HIV-positive and acquired immunodeficiency syndrome (AIDS) patients is unknown, estimates vary from 15 to almost 50% of such individuals. The type of neuropathy or myopathy related to the specific stage of HIV infection, the pathogenetic mechanisms involved, and effective therapies are discussed. A neuromuscular disease not only occurs in patients with AIDS and AIDS-related complex, but it can coincide with HIV seroconversion or it can be the only clinical indication of a chronic silent HIV infection. Chronic asymptomatic HIV infection should be considered in the differential diagnosis of certain acquired inflammatory polyneuropathies or myopathies. Precautions needed when doing electromyographic studies are discussed.