Children with multisystem - Langerhans cell histiocytosis (MS-LCH) that fails to respond to conventional chemotherapy have poor outcomes. Hematopoietic stem cell transplant (HSCT) represents a potential salvage approach. We report a first case of successful haploidentical transplantation in one child with refractory MS-LCH using T cell replete graft and post transplant Cyclophosphamide (PT-CY) from father as a donor. On last follow up he is 21 months post transplant with no signs of disease activity. This case report suggests that T-cell replete haploidentical donor transplantation using PTCY is a feasible and successful curative option for Patients with refractory MS-LCH who have no matched donor.