Observation:
En coup de sabre is a localised collagen vascular disorder which may sometimes be associated with deeper involvement of underlying neurological involvement. It is multifactorial in causation and genetic predisposition has a definite role to play. A classical case of en coup de sabre is described in a 13 year old boy with interesting neuropychiatric associations. The presence of similar lesions in his mother makes the case even more interesting. Though psychiatric associations are less commonly described with en coup de sabre, it should be looked for. The familial occurrence highlights the genetic and probably environmental causation of this disease.
Keywords: Familial en coup de sabre, neuropsychiatric manifestations