Neurological heterotopic ossification (NHO) involves the formation of bone in soft tissue following a neurological condition, of which the most common are brain and spinal cord injuries. NHO often forms around the hip, knee and shoulder joints, causing severe pain and joint deformation which is associated with significant morbidity and reduced quality of life. The cellular and molecular events that initiate NHO have been the focus of an increasing number of human and animal studies over the past decade, with this work largely driven by the need to unearth potential therapeutic interventions to prevent the formation of NHO. This review provides an overview of the present understanding of NHO pathogenesis and pathobiology, current treatments, novel therapeutic targets, potential biomarkers and future directions.