Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by degeneration of upper and lower motor neurons, leading to muscle weakness, spasticity, dysarthria, and dysphagia. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune-mediated neuropathy that primarily affects nerve fibers specifically myelin sheets. Clinically, CIDP presents with distal muscle weakness, prominent sensory disturbances, and diminished or absent deep tendon reflexes. The co-occurrence of ALS and CIDP is exceptionally rare and poses significant diagnostic and therapeutic challenges due to overlapping and distinct clinical features. A Case: A 44-year-old male presented to the Department of Neurology at the Mongolia-Japan Hospital, Mongolian National University of Medical Sciences, with progressive muscle weakness in both upper and lower extremities, along with dysphagia, especially for solids with frequent choking episodes. The initial symptoms began in May 2023 with muscle fasciculations, followed by progressive weakness, initially in the right upper limb and gradually progressing to the left. By August 2023, the patient developed bilateral arm weakness, dysarthria, and worsening dysphagia. From August 2024, episodes of head drop were noted. A progressive weight loss of 11 kg was recorded since January 2024. Comprehensive neurological evaluation, including antibody profiling, electromyography (EMG), and nerve conduction studies (NCS), supported a diagnosis of amyotrophic lateral sclerosis with chronic inflammatory demyelinating polyradiculoneuropathy-like neuropathy. Outcome: One month after hospital discharge, the patient demonstrated improvement in self-care abilities and increased muscle strength in both proximal and distal upper limb muscles. Notably, there was marked improvement in overall clinical status. Conclusion To our knowledge, this is the first reported case in Mongolia documenting the simultaneous presentation of ALS and CIDP-like neuropathy. Globally, such cases are exceedingly rare. Timely and accurate diagnosis, along with appropriate treatment, contributed to improved clinical outcomes and a deceleration of disease progression in this patient.