Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.

Secondary cutaneous dissemination from an orbital diffuse large B cell lymphoma has not been described before. The authors report an unusual case of anaplastic variant of diffuse large B cell lymphoma which primarily presented in the orbit and during the course of disease had subcutaneous dissemination.