Multiple carboxylase deficiency is biotin-responsive disorder. Affected patients present with metabolic acidosis, hypotonia, lethargy, skin rash, alopecia and seizure. The symptoms can manifest soon after birth or late in the infantile period. The prevalence is unknown. However, it is considered a rare disorder. It is inherited in an autosomal recessive pattern. Treatment includes a supplementation of biotin 5-40mg/day. An early institution of biotin ensures a better outcome.