Orientation to paraphenylenediamine (PPD) acute systemic intoxication in Egypt has been increased over the last decade. The aim of this study was to provide more insight into the clinical profile of acute PPD intoxication with reviewing the possible underlying mechanisms. Our study was retrospective. It was conducted over 7 years (2001-2008) on 25 cases with acute PPD intoxication admitted to the Poison Control Center Ain Shams University Hospitals, Cairo, Egypt. The mean age of the cases was 35.34 +/- 10.5 years; the male to female ratio was 18:7. Cervicofacial and laryngeal edema was the dominating presenting manifestation in 72% of the cases, 100% of the cases developed rhabdomyolysis, 80% had impaired renal functions, elevated liver transaminases were detected in 76% of cases, 75% showed hyperkalemia and 16% died due to ventricular arrhythmia. In conclusion, PPD causes serious multisystem toxicity and its selling to the public should be officially restricted.