Introduction

Xeroderma Pigmentosum is an uncommon genetic condition that is characterized by sensitivity to ultraviolet rays, ultimately leading to severe sunburn reactions and increased risk of skin cancer (1). Patients require stringent photoprotection measures, including sunscreen application and protective clothing (2). The clinical manifestations include hypo and hyperpigmented macules, premature aging, sunburns, blistering, and increased incidence of different malignancies in the skin. It also affects the eye and central nervous system (3). Patients involved in the eye can experience progressive blindness (6). A complex relationship exists between Xeroderma Pigmentosum and other malignancies, which is poorly understood (2). Priya and Janaki (2017) reported rare cases of carcinoma of the esophagus associated with xeroderma pigmentosum (4). Though susceptible to different cancers, few cases have been reported till now due to its rare nature (5). There is an association with neurological symptoms in 20 % of cases (6).  Herein, we present the case of a 22-year-old male diagnosed with Xeroderma Pigmentosum who developed Spindle Cell Carcinoma and experienced focal seizures. This case report is the first documented case associating Xeroderma Pigmentosum, Spindle Cell Carcinoma, and focal seizures.