Organic acidurias are suggested to be the most common lifethreatening inborn error of metabolism. Early diagnosis and treatment canimprove the outcome and survival in organic acidurias. This retrospectivestudy included 16 cases diagnosed as organic Acidurias. The specificdiagnosis of this 16 cases are : maple syrup urine disease (3 cases),alkaptonuria (4 cases), methylmalonic acidemia (2 cases), biotinidasedeficiency (2 cases), propionic acidemia (one case), isovaleric acidemia(one case), glutaric acidemia (one case), Hydroxymethyl glutaryl (onecase), beta- ketothiolase deficiency (one case).These patients was subjected to full clinical evaluation including history and afull clinical examination and several laboratory investigations. Results showedthat, Seizures was the main presenting symptom followed by vomiting and darkdiscoloration of urine. Moreover, disturbed conscious level was observed in50% of cases.The aim of the work is to study the pattern of clinical presentationand laboratory finding in patients of organic aciduria and the cliniciansmust be aware by the organic acidurias because outcome is enhanced bydiagnosis in the first ten days of life.