Aim: This case report aims to highlight the importance of preoperative histopathology of ambiguous cutaneous lesions of the ear, nose, and throat. Background: Epidermal inclusion cyst represents the most frequently observed benign cutaneous lesion. It typically presents as a smooth, unilocular, protruding, spherical, dome-shaped papule or nodule beneath the skin. It is generally diagnosed based on its clinical appearance, but histopathology is mandatory to confirm the diagnosis. It is treated by surgical excision. We report a rare case of clinical misdiagnosis of multiple benign epidermal inclusion cysts on the nose as rhinophyma. The clinical features of these cysts had striking similarities with rhinophyma, and hence it was treated the same without prior histopathology. Such a case of benign epidermal inclusion cysts having resemblance with rhinophyma would be reported for the first time in medical literature through this case report. Case presentation: We are presenting the case of a 65-year-old male, resident of Karachi and fishmonger by profession who presented to the otorhinolaryngology department of Dr. Ruth K. M Pfau Civil Hospital, Karachi, in April 2021, with the complaint of two bulbous swellings on the lower two-thirds of the dorsum of the nose with broadening of the nose for 12 years and nasal obstruction for 6 months. The swellings had appeared gradually and progressively increased in size. The past history and family history of the patient were insignificant. On compression, there was oozing of scanty, yellowish material from the pits of the lesion on dorsum nasi. The swellings were firm, non-compressible, non-fluctuant, and non-tender, with regular margins and open pores. Clinical features suggested a strong diagnosis of rhinophyma. After thorough investigations, it was decided to surgically excise the lesion. Without preoperative histopathology and sole reliance on clinical features, it was treated as rhinophyma by surgical excision and electrocautery and left for spontaneous re-epithelialization. However, postoperative histopathology revealed multiple benign epidermal inclusion cysts. The treatment yielded promising cosmetic and functional outcomes with no complications. Conclusion: The treatment received by the patient proved beneficial and yielded excellent results. The patient recovered, his symptoms were resolved, and a good cosmetic recovery was possible. Clinical significance: It is important to maintain that treatment of ambiguous cutaneous lesions involving the ear, nose, or throat should not be commenced with insufficient proof or sole reliance on clinical features. Also, our case highlights the importance of histopathology for differentiating seemingly identical lesions.