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Abstract Amyloidosis, a disease with extracellular tissue deposition of fibrils, results in clinical manifestations based on deposition of these fibrils in multiple organ systems. Usual manifestations include nephrotic‐range proteinuria, cardiac failure, hepatosplenomegaly, and skin manifestations. Common neurological manifestations include peripheral and autonomic neuropathies. Cranial neuropathy has been seldom reported and is an unusual clinical feature of amyloidosis. Here, we report an older man who presented with cranial nerve palsies along with other clinical features, including heart failure, proteinuria, weight loss, anorexia and distal symmetric polyneuropathy and was diagnosed with immunoglobulin light‐chain (AL) amyloidosis.