ABSTRACTUnilateral focal neuromyotonia has been rarely reported in fingers or extraocular muscles. We report a case of segmental neuromyotonia in a 20-year-old boy who presented to us with intermittent tightness in right upper limb. Electromyography revealed myokymic and neuromyotonic discharges in proximal as well as distal muscles of the right upper limb. Patient’s symptoms responded well to phenytoin therapy. Such an atypical involvement of two contiguous areas of a single limb in neuromyotonia has not been reported previously. Awareness of such an atypical presentation of the disease can be important in timely diagnosis and treatment of a patient.