Case Report A 33-year-old white female, had a history of progressive loss of vision and hearing since the age of 20, cardiomyopathy, cardiac arrhythmias, gastroparesis, urge incontinence, decreased bone mass, bilateral hip replacement, multiple surgical interventions on the right ankle, and one surgical intervention on the right knee. At the age of 27, she was diagnosed with Refsum disease by associating the shortening of the 4 th metatarsals bilaterally and the retinitis pigmentosa, along with a high phytanic acid level. She was started on the appropriate diet consisting of restrictions on green vegetables, meats from ruminating animals, and dairy products. Since the age of 30, the patient was hospitalized four times at another facility for acute decompensation of her disease. During each hospitalization, she underwent five sessions of plasmapheresis. During her last hospitalization, her phytanic level was 105.5umol/L (reference range : ≤ 9.88). It decreased to zero after the completion of four sessions of plasmapheresis. She took ibuprofen 800 mg by mouth every eight hours during her hospital stay at the other facility. The patient presented to the emergency department at our facility for acute exacerbation of her disease, including retroorbital headaches, chest pain, nausea, vomiting, diarrhea, urinary incontinence, diffuse muscle aches, and weakness that had increased in intensity over two weeks. A detailed history revealed that she was compliant with her diet, but had a chronic ibuprofen intake for recurrent tension headaches. She was never told that ibuprofen is contraindicated in Refsum disease. The patient improved significantly after five sessions of plasmapheresis and discontinuation of ibuprofen.