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Amyotrophiclateralsclerosis(ALS)isaprogressiveneurodegenerativediseaseofacentralnervoussystemthatischaracterizedbyisolatedcelldeathofbothupperandlowermotorneurons.OntheadvancedstagesofthediseaseALSdiagnosisisclear.ButontheearlystagesALScanmimicotherneurologicaldisordersandcausediagnosticpitfalls.DifficultieswithdiagnosisusuallyoccurincaseofcervicothoracicALSformifthereisaflaccidupperparalysis.ThereisagroupofthediseasesthatcanmimiccervicothoracicALS,amongothersmonomelicamyotrophyhasbeendescribed.MonomelicamyotrophyalsoknownasHirayamadisease(HD)isarareformofcervicalmyelopathy,itischaracterizedbymostlyunilateralorasymmetricalbilateralmuscularatrophyandweaknessoftheforearmsandhandsmanifestinginearlyadulthood.Thediseaseissupposedtoappearduetoabnormalanteriaddisplacementofthecervicalduralsacposteriorwall.Suchdeformationleadstolowercervicalcordcompressionandchronicischemiaofspinalcord’greymatter.TheHDincidenceisrare,malepredominancehasbeenreported. Weherebypresentacaseofthedisease,firstlydiagnosedasALS.