Objective To study the clinical features of different types of Miller-Fisher syndrome (MFS), and to analyze atypical clinical manifestations of MFS. Methods The clinical data of 19 children with MFS were retrospectively analyzed. Results Among the 19 children (7 boys and 12 girls), 10 had classic MFS, 1 had acute ophthalmoparesis (incomplete MFS), 2 had MFS/Bickerstaff brainstem encephalitis (BBE), and 6 had MFS/ Guillain-Barré syndrome (GBS). Nineteen children had the Ⅲ, Ⅳ and Ⅵ cranial nerve palsy, and three children had ophthalmoplegia. There were 7 cases of cranial nerve palsy other than ophthalmic nerve, including 5 cases of Ⅶ cranial nerve palsy, and 5 cases of Ⅸ and Ⅹ cranial nerve palsy. The atypical symptoms included visual deterioration in 2 cases, proptosis in 1 case, ophthalmalgia in 1 case, upper eyelid swelling in 1 case, dizziness in 2 cases, and headache in 1 case. Eight patients had positive peripheral neuropathy antibodies including GQ1b-IgG, GQ1b-IgM, GD1b-IgM, GD2-IgM, GT1a-IgG, GM1-IgG, GM2-IgM, Sulfatide-IgG, and Sulfatide IgM+IgG. One patient had positive myasthenia gravis antibodies (acetylcholine receptor antibody and ryanodine receptor calcium release channel antibody). Finally, MFS was diagnosed after full differential diagnosis combined with clinical practice. Conclusions Different types of MFS have different characteristics, and there are atypical symptoms. Antibodies should not be used as diagnostic criteria alone, and comprehensive clinical analysis should be combined.