The pseudopolyneuritic form of amyotrophic lateral sclerosis (ALS) is a subtype of ALS characterized by distal weakness of the lower limb and absence of Achilles tendon reflex. Moreover, this form could be presented with foot drop. Therefore, the diagnosis of this form of ALS is important for clinicians because the clinical findings could indicate peripheral neuropathy. The aim of this study was to define the significance of the pseudopolyneuritic form of ALS with foot drop. We analyzed the clinical records of 138 (males, n=83; females, n=55) ALS patients who were admitted to the Department of Neurology between January 1995 and September 2011. Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised was used to quantify the patients. Onset complaints were in the upper extremity (n=73), lower extremity (n=33), bulbar involving (n=25), dizziness (n=3), unilateral paresthesia, and arm pain (n=4). Among the patients with lower-limb onset, in 4 patients, the onset of lower-limb weakness was associated with distal dominance and absence of Achilles tendon reflex with foot drop. All these patients were male. In additional, 2 of the 4 patients are still alive, 1 of the patients died because of lymphoma and the other died because of respiratory failure. This form of ALS had better survival than other phenotypes. Our findings underline the clinical importance and correct diagnosis for this form of ALS with foot drop.