Gorlin–Goltz syndrome (GGS), also known as Nevoid basal cell carcinoma syndrome (NBCCS), or Gorlin syndrome, is a rare autosomal dominant inherited genodermatosis characterized by multiple nevoid basal cell epitheliomas at an early age, multiple jaw cysts, bifid ribs, and a multisystem developmental abnormality mostly affecting the skin, jaw, and central nervous system. Additionally, it includes a predisposition to neoplasms. The estimated prevalence varies from 1/57,000 to 1/256,000, without gender predominance. Cases have been reported from many countries except Yemen. We present a 42-year-old male patient who has presented with a coarse face, frontal bossing, synophrys, mandibular prognathism, multiple BCCs from the age of 15 years, palmoplantar pits, bifurcation and splaying of the left third rib, odontogenic keratocysts of the mandible, and calcifications of both the falx cerebri and the tentorium cerebelli. Additionally, he showed coloboma of the left lower eyelid and a caféau-lait (CAL) macule over the right waist area. He was diagnosed with Gorlin syndrome based on the presence of four major and four minor diagnostic criteria. Our case is unique as the patient presented with coloboma of the left lower eyelid, which has never been reported before, and a CAL macule, which has been reported only three times previously in association with Gorlin syndrome.