A fracture is defined as pathological when it arises in a bone tissue that has been modified and reshaped by a local or systemic pathological process. In children, pathological fractures can be secondary to several conditions, ranging from metabolic diseases to tumors, infections or neuromuscular pathologies. History, clinical examination and radiologic assessment are essential to making a diagnosis, to identifying the underlying cause and to planning the right treatment of a pathological fracture. Treatment must be tailored to both the fracture and the underlying cause. The objective of this work is to present the diagnostic approach and the course to follow when a child presents with a pathological fracture. The most common causes of pathological fractures, as well as their characteristics, will be described. Pathological fractures occurring in osteogenesis imperfecta and in abused children as well as stress fractures will not be discussed.