Pigmented epithelioid melanocytoma (PEM), a heavily pigmented and highly uncommon melanocytic lesion, is extremely rare. It comes under several forms and names. We report the case of a three-year-old boy born with an isolated right lumbopelvic and femoral giant PEM. The boy was unable to walk due to the size and the shape of the lesion. He underwent two stages of intra lesional resections, one in Benin and the second in Switzerland. The first surgery was followed by hypothetic hypovolemic and anaphylactic shocks, and the second surgery by a continuous lymphatic leakage from the wounds for months. The strategic management approach, the surgery and follow-up of this case of giant PEM presented a real challenge. (C) 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license