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Case series Patient: Male, 26 • Female, 21 Final Diagnosis: Umbilical pilonidal sinus Symptoms: Hair tuft in the umbilicus • pain • periumbilical dermatitis • purulent discharge from the umbilicus • skin lesions • pruritis Medication: — Clinical Procedure: Umbilicus preserving surgery Specialty: General Surgery • Dermatology • Plastic Surgery Objective: Rare disease Background: Umbilical pilonidal sinus (UPS) is a rare disease of young, hirsute, dark men with deep navels and poor personal hygiene. UPS could easily be misdiagnosed and mistreated due to its rarity and lack of awareness of the condition by physicians. However, the diagnosis is easy to establish with physical examination and a detailed history. Although it is being diagnosed and reported more frequently, there is still no consensus regarding best treatment options. Case Report: In this report, we present two cases of UPS, one in a man and one in a woman, who had typical symptoms of pain, swelling, and intermittent malodorous discharge from the umbilicus. They had small sinus openings with hair protruding deep in the navel. Because these two patients had previous histories of failed conservative treatments, an umbilicus preserving surgery was performed for both cases. Wounds were healed in 2–3 weeks with acceptable cosmetic results. During a more than 2 year follow-up period, there were no signs of recurrence. Conclusions: In a patient presenting with a history of intermittent discharge, itching, pain, or bleeding from the umbilicus and the presence of granulation tissue with or without protruding hair and periumbilical dermatitis, the diagnosis should consider UPS, even in female patients. Treatment generally depends on the severity of the disease, ranging from good personal hygiene to surgical excision of umbilical complex. The treatment of choice for chronic intermittent cases is surgical removal of the affected portion; paying special attention to cosmetic appearance.