Congenital kaposiform hemangioendothelioma: don't let the appearances fool you

Objectives: To describe challenges in diagnosis and treatment of congenital Kaposiform hemangioendothelioma (KHE). Case presentation: The neonate was born with an upper extremity vascular tumor and stable clinical state. His biopsy was inconclusive before discharge. At three weeks follow-up he presented with Kasabach-Merritt phenomenon (KMP), leading to the diagnosis of KHE, a rare locally aggressive vascular tumor that was managed with combination of steroids and mammalian target of rapamycin inhibitor (mTOR). Conclusions: KMP in combination with the lesion biopsy can enhance KHE diagnosis, leading to successful treatment with mTOR inhibitor.