Introduction: Acute hemorrhagic edema of infancy is an acute and rare cutaneous disorder that affects children between 4 months to 2 years of age and characterized by cutaneous purpuric lesions with millimeters to centimeters diameter. Systemic involvement is rare. The disease is benign and spontaneously resolved in 1-3 weeks.

Case Report: Here we described a 22-months old girl with acute hemorrhagic edema of infancy who hospitalized in department of pediatric with the presence of erythematous-purpuric lesions localized on the face, ears and lower limbs that developed suddenly two days before hospitalization. Laboratory examination revealed including normal cell blood counts, serum complements, serum electrolytes and creatinin. ANA was negative. Coagulation tests were normal. ESR was 45 mm/h. Urine analysis and stool examination revealed no abnormal findings. Disease resolved spontaneously without any problem.

Conclusion: According to the contrast between the acuteness of the cutaneous sign, which are typical and unmistakable, and the general condition of the patient, which was good and the laboratory findings, our patient labeled as acute hemprrhagic edema of infancy.