Introduction: Systemic capillary leak syndrome (SCLS) is a rare disease characterized by recurrent episodes and a triad of “leak attacks”: hypovolemic shock, generalized edema, hemoconcentration and paradoxical hypoalbuminemia. Case study and discussion: Here we report a case of pediatric idiopathic SCLS with an episode of cough and fever followed two days later by myalgia, livedo, acrocyanosis, and five days later by edema, tachycardia, hypotension, and generalized tonic-clonic seizure. Moreover, we provide evidence for an LPS-induced overproduction of interferon-gamma and interleukin-17 by the patient's peripheral blood mononuclear cells one year after the attack. Conclusion: This observation suggests the involvement of IL-17 in the pathogenesis of this disease.