Background <p>Cylindroma is a benign adnexal tumor mostly found on the scalp or face. They present as pink to purple, smooth-surfaced, solitary or multiple nodular lesions, mostly affecting middle-aged and elderly women. Reports of solitary cylindromas occurring in other anatomical locations are rare.</p> Case presentation <p>We present a unique case of a 56-year-old Arab male patient with a solitary, large, non-ulcerated cylindroma on the left-sided neck. The lesion had progressively enlarged since childhood, with worsening symptoms such as pain and a burning sensation over the last 2 years. A second, smaller cystic lesion was found behind the left ear. The patient had no significant family or past medical history, and specifically no history of skin diseases. Surgical excision of both lesions was performed. Histopathology confirmed a benign dermal cylindroma and a keratinous epidermal cyst. The lesion demonstrated characteristic histological features: irregular basaloid islands in a jigsaw pattern surrounded by hyaline basement membranes. Postoperative recovery was uneventful.</p> Conclusion <p>This case highlights a rare clinical presentation of a large benign cylindroma in an unusual location. Surgical excision remains an effective and definitive treatment.</p>