ABSTRACT
Peripheral neuroepithelioma is a rate malignant tumour of primitive peripheral non autonomic nervous system. The tumour can occur at any age involving soft tissue mainly of lower extremities. Other sites of occurrence are the shoulder-thoracic region and the head and neck. It is a very aggressive tumour with early metastasis mainly to the lung. Herewith we report a case of 16 years old female who was presented with soft mass of labia majora of 3 years duration which has been rapidly enlarging in the last 6 months. The mass was excised and followed by administration of chemotherapy and radiotherapy. The patient developed secondaries in the lung 3 months after excision, with pleural effusion, but no local recurrence. However, eight months after the diagnosis and in January 2005, the patient died from the tumour