Amyloidosis is a disease which can affect any tissue in the body with extracellular deposition of low molecular weight protein fibrils. Amyloid deposition can appear in many organs and tissues. Amyloidosis of tongue occurs most commonly in systemic form as rubbery macroglossia due to extra-deposition of amyloid within the suprahyoid muscle. Anesthesiologist can experience the negative consequences of amyloidosis in many conditions. Difficult airway due to amyloid deposition in tongue is an unexpected condition. We aimed to report a case that had respiratory distress with extremely big tongue due to amyloidosis. We performed a fiberoptic intubation for emergent tracheostomy without any complication.