Denys-Drash syndrome.

In 1967, Denys et al. described the triad of ambiguous genitalia, nephrotic syndrome and Wilms' tumor in an XX/ XY mosaic(l). Three years later, Drash et al. described the triad in two patients and suggested that it may be a syndrome(2). Since then the syndrome has come to be known as the Drash syndrome or, more appropriately, as the Denys-Drash syndrome. More than 60 patients with this syndrome have been reported(3). We report on a child with this syndrome to highlight the issues that made the diagnosis difficult in our case.