We illustrate the case of a 71-year-old male who initially presented with sudden onset muscle weakness and ambulation difficulty. Following medication discontinuation and additional clinical studies, he failed to improve and was admitted to the hospital 11 weeks later. He had an associated 20-pound weight loss, sudorrhea, and muscle stiffness only when weight-bearing. A complete connective tissue cascade and a paraneoplastic panel were obtained. Clinical diagnosis of acquired neuromyotonia, or Isaacs syndrome (IS), was made, and he began experiencing significant improvement after intravenous steroid infusion. IS is a rare disease that has been poorly documented in the literature. There have only been a limited number of cases which are globally documented. One of the difficulties is a lack of definite autoantibody with which to correlate the disease; however, there has been some correlation linking the disease to voltage-gated potassium channels. Ultimately, the diagnosis should be driven by history and clinical presentation. The aim of this case report is to highlight a rare disease process and increase awareness among clinicians. We also describe the associated evaluation and recommended treatment for an optimal patient outcome.