Umbilical pilonidal sinus is a rare diagnosis which is characterized by an inflammatory granulomatous reaction to hair shafts penetrating the epidermis. It is most often seen in adolescent male with a hairy abdomen. The patients often present with a history of pain and umbilical discharge. Conservative treatment with hair extraction and personal hygiene is prioritized and surgery is only recommended in recurrent cases. Here one such case is presented, which was resistant to conservative treatment and where surgical excision and primary repair was indicated. No recurrence was observed 6 months postoperatively.