Acute motor axonal neuropathy (AMAN) is a variant of Guillain-Barré syndrome (GBS), characterized by acute areflexic flaccid quadriparesis with motor axonal changes and absence of demyelinating findings in electrophysiological studies. A 30-year-old man presented with acute onset flaccid type of weakness involving all four limbs, along with drooping of eyelids. Examination revealed ptosis with restricted horizontal and vertical eye movements. Spinomotor system examination revealed acute flaccid areflexic quadriparesis. Nerve conduction studies (NCS) showed features suggestive of motor axonal neuropathy changes. Cerebrospinal fluid (CSF) revealed albuminocytological dissociation. The diagnosis of AMAN was made, and the patient was treated with intravenous immunoglobulin (IVIg). His weakness gradually improved over 1 month, with partial improvement in ptosis and eye movements. This case highlights the occurrence of ophthalmoparesis in the AMAN variant of GBS. The presence of ophthalmoparesis and areflexia makes it necessary to exclude Miller-Fisher syndrome. But, the presence of axonal changes in nerve conduction study and the profound weakness with negative serum anti-GQ1b antibody profile, supports the diagnosis of AMAN. How to cite this article: Budumuru U, Muralidharan K, Sowmini PR, et al. AMAN with Ophthalmoparesis: A Rare Presentation. J Assoc Physicians India 2023;71(11):103-104.