Introduccion: el onfalocele es una malformacion que puede presentarse aislada o como parte de un sindrome. Aunque es esporadico, se han informado casos de herencia familiar. La mayoria de los casos de onfalocele son esporadicos y el riesgo de recurrencia es menor del 1%. No hay factores etiologicos especificos. En estudios de poblacion se ha encontrado mayor incidencia entre las madres mas jovenes, por lo cual no se pueden tomar medidas preventivas eficaces. Ante la presencia de onfalocele, deben buscarse otras anomalias congenitas. Caso clinico: paciente con embarazo de 16 semanas y feto con presentacion atipica de onfalocele, que hizo sospechar la existencia de sindrome de Prune-Belly, por la presencia de un quiste grande en pared abdominal. El diagnostico definitivo fue establecido por anatomia patologica. Cariotipo fetal normal. Conclusiones: sin que se hayan determinado factores etiologicos definitivos para el mismo, el onfalocele puede presentarse como un sindrome, por lo que deben buscarse anomalias congenitas asociadas. Rev.cienc.biomed. 2012;3(2):355-359 PALABRAS CLAVES Onfalocele; Gastrosquisis; Pared abdominal; Malformaciones congenitas; Sindrome de Prune-Belly SUMMARY Introduction: omphalocele is a malformation that can be presented isolated or as part of a syndrome. Although it is sporadic, cases of familiar inheritance have been informed. The majority of the cases of Omphalocele are sporadic and the risk of recurrence is less than 1%. There aren´t specific etiologic factors. In studies of population, greater incidence has been found between younger mothers, for what effective preventive measures can´t be taken. In view of the presence of Omphalocele, others congenital anomalies should be searched. Clinical case: patient with 16-weeks pregnancy and fetus with atypical presentation of Omphalocele that caused suspicion of the existence of Prune-Belly Syndrome due to the presence of a large cyst in abdominal wall. The definitive diagnosis was established by pathological anatomy. Fetal karyotype was normal. Conclusion: without definitive etiologic factors had been determinated for omphalocele, it can be presented like a syndrome, for what associated congenital anomalies should be searched. Rev.cienc.biomed.2012;3(2):355-359 KEY WORDS Omphalocele; Gastroschisis; Abdominal wall; Congenital malformations; Prune-Belly syndrome.