Silver syndrome, or distal hereditary motor neuropathy type V, is a rare dominantly inherited disorder caused by mutations in the BSCL2 gene. Silver syndrome causes a characteristic pattern of distally-predominant upper extremity weakness and wasting, often in association with long-tract signs in the lower extremities. We present a case of Silver syndrome presenting with rapidly progressive weakness and wasting of the hand musculature in a 13 year-old boy. Examination revealed marked distal wasting of his hands, with less prominent wasting of the calves and early pes cavus. Sensation was intact and deep tendon reflexes were universally brisk. Nerve conduction studies were consistent with a moderately severe sensorimotor axonal neuropathy pattern. Genetic testing revealed the common S90L mutation in the BSCL2 gene.