Background: Distal/asymmetric presentations of myasthenia gravis (MG) are uncommon and occur in 3-7% of patients with MG. This pattern of weakness is often not recognized as a manifestation of MG, leading to inappropriate investigations, delayed diagnosis and potentially missed opportunities for treatment. Our knowledge about this atypical presentation is limited to small case series and individual case reports. This study therefore aims to expand our understanding by describing the clinical course, diagnosis and treatment of a larger series of patients with this presentation. Methods: We conducted a retrospective chart review of patients with definite MG (either acetylcholine receptor [AChR] or MuSK antibody positive or clear evidence of postsynaptic neuromuscular junction dysfunction on electrodiagnostic studies), who attended the MG Clinic in London. Details of the clinical course, electrodiagnostic studies, antibody testing and response to treatment are reported. Results: 5.9% (54/921) of patients with definite MG had distal/asymmetric limb involvement, 56% at onset and 4% developing more than 10 years later. Males predominated (2:1). Finger extensors were most affected. 83% were AChR antibody positive. 7% had thymomas. On repetitive nerve stimulation most patients showed the most significant decrement distally on the more affected side. Almost all patients improved with treatment. Conclusions: This study expands our understanding of distal/asymmetric presentations of MG.