Michelin tire baby syndrome (MTBS) is a benign hamartomatous condition with ring-like lesions present on the limbs and trunk. MTBS is a rare genodermatosis. According to our search, only 20 cases have been reported. We present a case of a six-month-old female child, with complaints of fever and seizures. Since birth, she had asymptomatic multiple, asymmetric skin folds on all four limbs, resembling “Michelin Man” logo of the French tire manufacturer. She had microcephaly with characteristic round face hypertelorism, depressed nasal bridge, hypertrichosis with low set ears, a thin down-turned vermillion border of the upper lip, and a short neck. MRI was normal. Clinically, the diagnosis of MTBS was made. In addition, the parents were counseled about the self-limiting course of this disorder. MTBS itself might not be a single disorder but may manifest as a clinical finding associated with other disorders; therefore, a regular follow up of these patients is usually advised.