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

Abstract 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.