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Purpose Chronic progressive external ophthalmoplegia (CPEO) is a rare mitochondrial myopathy characterized by slowly progressive paralysis of the extraocular muscles with onset around the age of 20. Patients usually experience bilateral ptosis before developing symmetric, multidirectional external ophthalmoplegia. Methods 31-year-old woman with 10 year history of slowly progressive paralysis of the extraocular muscles was referred to our institute. Every action of extraocular muscles was reduced in both eyes. She could not adduct and elevate, there was only a slight abduction and depression function in both eyes. Patient had no ptosis or exophthalmus. BCVA was 20/20 in both eye. Slit lamp examination was normal. Dilated funduscopic examination revealed slight pigment disturbance in the periphery. Visual field and color vision tests were normal. Electroretinography showed lower borderline scotopic b-wave amplitudes. Laboratory tests showed increased lactic acid and creatine phosphokinase values. The results of liver and kidney function, autoimmun and serologic tests were normal. All thyroid function tests yielded negative results. Urinalysis, chest x-ray, heart ultrasound, brain and orbital magnetic resonance, nasal sinus CT scans were normal. Tensilon test excluded myasthenia gravis. Results We considered the case as CPEO clinically, mitochondrial DNA analysis is in process. Conclusion In CPEO, slowly progressive paralysis of the extraocular muscles may develop without ptosis. A careful differential diagnosis is very important.