Herein, we present the case of a patient who had complaint suggestive of carpal tunnel syndrome due to fibrolipoma of the median nerve and macrodactyly. 32-year-old male patient has macrodactyly of the right index finger and coarse skin lines and hyperkeratosis on the volar aspect of the index finger. The patient reported numbness and tingling sensation in his right hand that have woken him at night for 10 years. Electromyography, magnetic resonance imaging (MRI) of the right hand and diagnostic biopsy were performed to the patient. Sensory action potential of the right median nerve was absent. The median motor study on the right showed low amplitude of the compound muscle action potential with a markedly prolonged distal latency. MRI scan demonstrated a lesion that extended from the palm to the forearm and gave cable-like appearance to the median nerve which is pathognomonic for lipofibroma. Biopsy result was consistent with fibrolipoma. Neural fibrolipoma is a rare benign tumor of the median nerve. MRI is the best method for observing the involvement of the nerve. In one-third of the cases, macrodactyly is associated with neural fibrolipoma. In the differential diagnosis of patients with carpal tunnel syndrome symptoms, fibrolipoma of the median nerve should also be kept in mind. (Archives of Neuropsychiatry 2011; 48: 155-7)