Canavan disease (CD) is caused by elevated levels of N-acetylaspartic acid (NAA). The triad of hypotonia, macrocephaly and head lag in infant after the age of three to five months should raise the suspicion of Canavan disease. The diagnosis of CD relies upon measurement of the concentration of NAA in the urine using gas chromatography-mass spectrometry (GC-MS). For certain diagnosis should be performed aspartoacylase enzyme activity in cultured fibroblast or molecular analyse of ASPA gene. Here presented a six months old girl patient applied with seizures to our clinic. In her physical examination lack of head control without macrocephaly and optic atrophy were found. We suspected neurometabolic disorder and performed metabolic screening tests. Urine GC-MS showed high level of NAA and magnetic resonance spectroscopy showed a peak of aspartic acid. After these results molecular analyse for CD were performed and confirmed the diagnose by the identified homozygous mutation (c.244dupA) in the ASPA gene. The first presentation of our patient were nonspesific neurological findings like seizures and hypotonia. Additionally there were no sign for macrocephaly which is generally seemed in CD.