RESUMENIntroduccion: el sindrome de Reye es una encefalopatia aguda asociada a una degeneracion grasa del higado que usualmente es precedida de una infeccion respiratoria o varicela y tiene una alta prevalencia en ninos menores de seis anos. Objetivo: reportar un caso clinico de sindrome de Reye congenito asociado a la infeccion por varicela adquirida de la madre. Presentacion de caso: se describen los hallazgos de la autopsia, la respectiva correlacion clinicopatologica de un recien nacido de sexo masculino de 37 semanas de gestacion, hijo de madre con varicela activa desde cuatro dias antes del parto, quien presento subitamente palidez generalizada, bradicardia y apnea. Resultados: el examen histopatologico encontro en el citoplasma de los hepatocitos y tubulos renales un compromiso vacuolar que correspondia a grasa. En el cerebro se evidencio severo edema, sin inflamacion perivascular o meningea. Conclusion: corresponde a un caso de sindrome de Reye congenito asociado a varicela materna, que termino manifestandose clinicamente como muerte subita. Podria ser la primera publicacion de un caso de sindrome de Reye congenito asociado a varicela materna. MED.UIS. 2014;27(3):113-121.Palabras clave: Sindrome de Reye. Varicela. Embarazo. Recien nacido. Anomalias congenitas.AbstractIntroduction: Reye’s syndrome is an acute encephalopathy associated with fatty degeneration of the liver that usually is preceded by a respiratory infection or chickenpox and is highly prevalent in children under 6 years old. Objective: to report a clinical case of congenital Reye’s syndrome associated with varicella infection acquired from the mother. Case report: we describe the autopsy findings with the respective clinicopathological correlation of a male newborn of 37 weeks of gestation, son of mother with active varicella from 4 days before birth, who presented sudden paleness, bradycardia and apnea. Results: histopathologic examination found in the cytoplasm of hepatocytes and renal tubules a vacuolar commitment that corresponds to fat. The brain showed severe edema without perivascular or meningeal inflammation. Discussion and conclusion: it corresponds a case of congenital Reye’s syndrome associated with varicella infection in pregnancy, who finished clinically as sudden death. This could be the first published case of congenital Reye’s syndrome associated with varicella infection in pregnancy. MED.UIS. 2014;27(3):113-121.Key Words: Reye Syndrome. Chickenpox. Pregnancy. Newborn. Congenital abnormalities.