Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a sensory and motor demyelinating polyneuropathy that occurs with autoimmune mechanisms, affects the spinal roots, plexuses, and peripheral nerves, and presents with attacks or progressivity. Proximal and/or distal strength loss and mild atrophy in the upper and lower extremities and sensory complaints, such as numbness, pain, and burning, are observed. Cranial nerve involvement rarely accompanies typical findings. Facial nerve involvement is the most common; however, trigeminal, oculomotor, hypoglossus, and abducens nerve involvements are reported. Multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) is an atypical variant of CIDP, characterized by asymmetrical motor and sensory losses and conduction blocks in the electromyography. Hashimoto’s thyroiditis is an autoimmune disease diagnosed with high serum thyroid antibody concentrations. Coexistence with neurological autoimmune diseases such as multiple sclerosis, myasthenia gravis, and multifocal motor neuropathy was reported. Moreover, few studies showed the coexistence of Hashimato’s thyroiditis and CIDP in the literature. Presented herein is a case of MADSAM with cranial nerve involvement and Hashimato’s thyroiditis.