Pilonidal sinus disease is a common problem of sacrococcygeal region. However, it is also observed in the periumbilical area. There are only a few reports about umbilical pilonidal sinus in the literature. In this study, 26 patients (24 men (92 %), 2 women (8 %) with a mean age of 22 years) with umbilical pilonidal sinus disease were included. Predisposing factors, patient characteristics, treatment modalities, and their results have been studied. Male sex, young age, hairiness, deep navel, and poor personal hygiene were found to be predisposing factors. Twenty-five patients were treated conservatively. However, two patients failed to respond to conservative treatment. Those patients underwent surgery where umbilectomy was carried out without reconstruction. One patient was also operated on for the preoperative misdiagnosis of irreducible umbilical hernia. Patients were followed for 14–96 months. We recommend conservative treatment in patients with umbilical pilonidal sinus. Surgery should be performed in recurrent cases resistant to conservative treatment. The importance of differential diagnosis of umbilical pilonidal sinus from other umbilical pathologies is also emphasized.