Tomaculous neuropathy (TN) is classically associated with the inherited, recurrent focal neuropathies. We report a case of TN manifesting as an acute recurrent polyneuropathy. A 28-year-old woman had 2 episodes of acute, ascending, symmetrical sensorimotor deficit. Teased nerve-fiber preparation confirmed the presence of TN. Extensive investigations failed to reveal other cause for her symptoms. We believe that this case is unique and broadens the clinical spectrum of TN.