The term "pseudoglioma" designates a heterogeneous group of diseases that may be confused clinically with retinoblastoma. The authors report the case of a one year old boy with leucocoria and exotropia of the left eye. Clinical examination disclosed a retrolental whitish vascularized mass. As a retinoblastoma could not be excluded with certainty, the blind eye was enucleated. Pathological examination showed a retinal vascular anomaly compatible with Coats' disease. The authors discuss the differential diagnosis of leucocoria and pseudoglioma.