A male neonate was born to a sixth-gravida mother with a history of four early-neonatal deaths. On day 21 of life, the patient was admitted for poor feeding, vomiting and encephalopathy. Final diagnosis of propionic acidaemica (propionylcarnitine, 17.67 μmol/L) was made. He was managed by peritoneal dialysis followed by protein-free and special lipid diet, sodium benzoate and multivitamins. On day 28 of life, he developed acrodermatitis enteropathica-like skin lesions on perioral and diaper area that did not respond to oral zinc or antimicrobials. A possibility of acrodermatitis acidaemica was kept and supplementation with essential amino acids started, following which the skin lesions regressed.