We report the case of an infant aged of 14 months deceased of sudden d
eath. The diagnosis of histiocytoid cardiomyopathy was made on a necro
psic basis. The pathologic examination showed a cardiac hypertrophy ch
aracterized by yellowish areas with irregular outlines, disseminated i
n the myocardium, and made of histiocyte-like cells with foamy or gran
ular cytoplasm. These cells reacted positively with desmin and myoglob
in labels, and had rare and disorganised myofibrils in electron micros
copy, proving their muscular origin. The illness affects infants and u
sually causes severe cardiac troubles leading to death without treatme
nt. This case is the fourteenth associated with sudden death.