Describes a case of xeroderma pigmentosum with symptoms like brownish yellow pigmentation of skin all over the body, atrophic spots in between the pigmented area, warty growths especially of the upper half of the body epidermoid carcinoma of the upper lip, right side of the face and left eye, rough and dry skin, photophobia, eye changes viz conjunctivitis keratitis, corneal opacities and loss of vision, fungated tumour into the eye ball ectropion of the left eye and histological changes in the skin. Role of heredity, some of the etiological factors and biochemical changes are also discussed.