Acute hemorrhagic edema of infancy (AHEI) is a benign, self-limiting vasculitis that usually resolves completely without any sequelae or a need for active therapy. To our knowledge, compartment syndrome because of AHEI has not been reported. Chart data for a single case were reviewed and reported in a retrospective study. A 19-month-old male presented with petechial rash and swelling of the left lower leg. AHEI was diagnosed clinically and confirmed by skin biopsy. On the basis of the clinical appearance, compartment syndrome of the foot was suspected. Measurements of compartmental pressures in the foot were well above the commonly cited ranges and a fasciotomy was performed. Following the operation, there was a marked clinical improvement in the limb perfusion. The child was discharged on the 20th day with marked clinical improvement; both active and passive leg movements were intact. We suggest that pediatric orthopedic surgeons should be familiar with this entity and its rare complication. Level of evidence: V – case report.