In the patients, no infectious agent was isolated from the cultures of various specimens. All the patients presented with metabolic acidosis and ketosis, but the fact that whether HLH was triggered by the toxic metabolites or vice versa is unclear. Whether the molecular basis of the diseases in the presented three cases serve as a determining factor is not known. Considering all these issues the mechanism playing a role in the development of hemophagocytosis in our three patients with organic acidemia is not clear yet. In patient 2 VH recurred once, with seizure, abnormal EEG, and brain MRI. Acute and chronic brain abnormalities are well-documented in PA. Acute or chronic focal cerebral metabolic or toxic insult as part of the PA might etiologically explain the VHs in our patients.